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TMPY-01104
|
Coagulation factor XI/F11 Protein, Human, Recombinant (His)
coagulation factor 11,coagulation factor XI,FXI
|
Human
|
HEK293 Cells
|
|
Factor XI (plasma thromboplastin antecedent) is a plasma glycoprotein, and a zymogen acting as a serine protease which participates in blood coagulation as a catalyst in the conversion of factor IX to factor IXa in the presence of calcium ions. It is an unusual dimeric protease, with structural features that distinguish it from vitamin K-dependent coagulation proteases. The factor XI is synthesized in the liver as a single polypeptide chain with a molecular weight estimated between 125 ~160 kDa ... |
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TMPY-02585
|
Acid sphingomyelinase/SMPD1 Protein, Mouse, Recombinant (His)
aSMase,ASM,Zn-SMase,A-SMase,sphingomyelin phosphodiesterase ...
|
Mouse
|
Baculovirus Insect Cells
|
|
Sphingomyelin phosphodiesterase 1 (SMPD1) , also known as ASM ( acid sphingomyelinase ), is a member of the acid sphingomyelinase family of enzymes. Three isoforms have been identified, isoform 1 is 631 amino acids (aa) in length as the pro form, while Isoform 2 and isoform 3 have lost catalytic activity. The active SMPD1 isoform 1 contains one saposin B-type domain that likely interacts with sphingomyelin, and a catalytic region. Human SMPD1 is 86% aa identical to mouse SMPD1. SMPD1 is a monome... |
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TMPY-02078
|
HtrA2/Omi Protein, Human, Recombinant (His)
PARK13,OMI,HtrA serine peptidase 2,PRSS25
|
Human
|
E. coli
|
|
Serine protease HTRA2, also known as high-temperature requirement protein A2, Omi stress-regulated endoprotease, Serine protease 25, Serine proteinase OMI and HTRA2, is a single-pass membrane protein that belongs to the peptidase S1B family. HTRA2 contains one PDZ (DHR) domain. HTRA2 is a serine protease that shows proteolytic activity against a non-specific substrate beta-casein. It promotes or induces cell death either by direct binding to and inhibition of BIRC proteins (also called inhibitor... |
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TMPY-01061
|
FGF-10 Protein, Human, Recombinant
fibroblast growth factor 10,KGF2
|
Human
|
E. coli
|
|
Fibroblast growth factor 10 (FGF10) is a member of the fibroblast growth factor (FGF) family. FGF family members possess broad mitogenic and cell survival activities, and are involved in a variety of biological processes, including embryonic development, cell growth, morphogenesis, tissue repair, tumor growth and invasion. FGF10 exhibits mitogenic activity for keratinizing epidermal cells, but essentially no activity for fibroblasts, which is similar to the biological activity of FGF7. FGF10 pla... |
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TMPY-02153
|
TNF beta Protein, Human, Recombinant
LT,TNF β,TNFSF1,肿瘤坏死因子,TNFB,lymphotoxin alpha,lymphotoxin α
|
Human
|
E. coli
|
|
Lymphotoxin-alpha, also known as LT-alpha, TNF-beta, Tumor necrosis factor ligand superfamily member 1, LTA TNFSF1, and TNFB, is a secreted protein that belongs to the tumor necrosis factor family. TNF-beta/TNFSF1/Lymphotoxin alpha is highly inducible, secreted, and exists as a homotrimeric molecule. It is a cytokine that in its homotrimeric form binds to TNFRSF1A / TNFR1, TNFRSF1B / TNFBR, and TNFRSF14 / HVEM. In its heterotrimeric form with LTB, TNF-beta/TNFSF1/Lymphotoxin alpha binds to TNFRS... |
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TMPY-02185
|
Coagulation factor XIII B/F13B Protein, Human, Recombinant (His)
Coagulation factor 13,coagulation factor XIII, B polypeptide...
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Human
|
HEK293 Cells
|
|
Coagulation factor XIII B chain, also known as Fibrin-stabilizing factor B subunit, Protein-glutamine gamma-glutamyltransferase B chain, Transglutaminase B chain and F13B, is a secreted protein which contains 1 Sushi ( CCP / SCR ) domains. Coagulation factor XIII is the last zymogen to become activated in the blood coagulation cascade. Plasma factor XIII is a heterotetramer composed of 2 A subunits and 2 B subunits. The A subunits have catalytic function, and the B subunits do not have en... |
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TMPY-02648
|
PACAP receptor/ADCYAP1R1 Protein, Human, Recombinant (hFc)
adenylate cyclase activating polypeptide 1 (pituitary) recep...
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Human
|
HEK293 Cells
|
|
Pituitary adenylate cyclase activating polypeptide (PACAP, Adcyap1) activation of PAC1 receptors ( Adcyap1r1) significantly increases excitability of guinea pig cardiac neurons. This modulation of excitability is mediated in part by plasma membrane G protein-dependent activation of adenylyl cyclase and downstream signaling cascades. Studies point to the potential role of the (pituitary) adenylate cyclase activating polypeptide receptor 1 (ADCYAP1R1) gene, which has been implicated in stress resp... |
|
TMPY-01380
|
Cyclophilin B Protein, Human, Recombinant (His)
CYPB,peptidylprolyl isomerase B (cyclophilin B),HEL-S-39,SCY...
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Human
|
HEK293 Cells
|
|
PPIB is identified as a candidate gene for OI-IX. Osteogenesis imperfecta (OI) is an inherited disorder of connective tissue typically caused by defects in either COL1A1 or COL1A2. A number of other genes causative of this disorder have been found, including PPIB, which forms one subunit of the prolyl 3-hydroxylase enzyme complex. Patients with OI caused by PPIB mutation should have appropriate early and regular management of their hearing. |
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TMPY-02394
|
IMPA1 Protein, Human, Recombinant (His)
IMPA,inositol(myo)-1(or 4)-monophosphatase 1,IMP
|
Human
|
E. coli
|
|
IMPA1 (Inositol Monophosphatase 1) is a Protein Coding gene. This gene encodes an enzyme that dephosphorylates Myo-inositol monophosphate to generate free Myo-inositol, a precursor of phosphatidylinositol, and is, therefore, an important modulator of intracellular signal transduction via the production of the second messenger's myoinositol 1,4,5-trisphosphate and diacylglycerol. Despite its many physiological functions, no clinical phenotype has been assigned to this gene dysfunction to date. Ad... |
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TMPY-00569
|
B3GAT3 Protein, Human, Recombinant (His)
beta-1,3-glucuronyltransferase 3,glcUAT-I,GLCATI,β-1,3-glucu...
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Human
|
E. coli
|
|
B3GAT3, encoding beta-1,3-glucuronyltransferase 3, has an important role in proteoglycan biosynthesis. Homozygous B3GAT3 mutations have been associated with short stature, skeletal deformities, and congenital heart defects. There is a novel B3GAT3-related disorder with craniosynostosis and bone fragility, due to a unique homozygous mutation in B3GAT3. |
|
TMPK-01376
|
TROP-2 Protein, Mouse, Recombinant (aa 25-270, His)
TACD2,gp50,TACSTD2,M1S1,TROP-2,GA733-1,T16,EGP-1,EGP1,TROP2
|
Mouse
|
HEK293 Cells
|
|
Trop-2,also known as epithelial glycoprotein-1 antigen (EGP-1),is a protein that in humans is encoded by the TACSTD2 gene.Mutations of this gene result in gelatinous drop-like corneal dystrophy, an autosomal recessive disorder characterized by severe corneal amyloidosis leading to blindness. |
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TMPH-03262
|
Cathepsin K Protein, Rat, Recombinant (His)
CTSO,CTSO2,Cathepsin O2,Cathepsin X,Cathepsin O,CTSK,Catheps...
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Rat
|
E. coli
|
|
Thiol protease involved in osteoclastic bone resorption and may participate partially in the disorder of bone remodeling. Displays potent endoprotease activity against fibrinogen at acid pH. May play an important role in extracellular matrix degradation. Involved in the release of thyroid hormone thyroxine (T4) by limited proteolysis of TG/thyroglobulin in the thyroid follicle lumen. |
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TMPY-00368
|
ALR Protein, Human, Recombinant (His)
HPO,ERV1,HERV1,ALR,HSS,HPO2,growth factor, augmenter of live...
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Human
|
HEK293 Cells
|
|
Alterations in GFER gene have been associated with progressive mitochondrial myopathy, congenital cataracts, hearing loss, developmental delay, lactic acidosis and respiratory chain deficiency in 3 siblings born to consanguineous Moroccan parents by homozygosity mapping and candidate gene approach. Using homozygosity mapping, we discovered that a mutation in the GFER gene causes an infantile mitochondrial disorder. |
|
TMPY-02377
|
14-3-3 eta/YWHAH Protein, Human, Recombinant (GST)
tyrosine 3-monooxygenase/tryptophan 5-monooxygenase activati...
|
Human
|
E. coli
|
|
YWHAH, the gene encoding the 14-3-3eta isoform, is highly expressed in retinal ganglion cells (RGC). YWHAH is a positional and functional candidate gene for both schizophrenia and bipolar disorder (BP). It is located on chromosome 22q12.3, a region that has been implicated by linkage studies in both BP and schizophrenia. |
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TMPY-00131
|
Niemann Pick C1/NPC1 Protein, Human, Recombinant (His & FLAG)
NPC,Niemann-Pick disease, type C1
|
Human
|
HEK293 Cells
|
|
Niemann-Pick C1 (NPC1), a host receptor involved in the envelope glycoprotein (GP)-mediated entry of filoviruses into cells, is believed to be a major determinant of cell susceptibility to filovirus infection. Niemann-Pick C1 (NPC1), a membrane protein of lysosomes, is required for the export of cholesterol derived from receptor-mediated endocytosis of LDL. The NPC1 protein is a multipass transmembrane protein whose deficiency causes the autosomal recessive lipid storage disorder Niemann-Pick ty... |
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TMPJ-01034
|
TIM Protein, Human, Recombinant (His)
TPI1,TPI,Triose-Phosphate Isomerase,Triosephosphate Isomeras...
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Human
|
E. coli
|
|
Triose-phosphate isomerase, also named Triose-phosphate isomerase, TPI and TIM, is an enzyme that catalyzes the reversible interconversion of the triose phosphate isomers dihydroxyacetone phosphate and D-glyceraldehyde 3-phosphate. TPI has been found in nearly every organism searched for the enzyme, including animals such as mammals and insects as well as in fungi, plants, and bacteria. However, some bacteria that do not perform glycolysis, like ureaplasmas, lack TPI. TPI plays an important role... |
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TMPY-04773
|
TPK1 Protein, Human, Recombinant (His)
THMD5,PP20,HTPK1,thiamin pyrophosphokinase 1
|
Human
|
E. coli
|
|
Thiamine pyrophosphokinase (TPK) produces thiamine pyrophosphate, a cofactor for a number of enzymes, including pyruvate dehydrogenase and 2-ketoglutarate dehydrogenase. Episodic encephalopathy type thiamine metabolism dysfunction (OMIM 614458) due to TPK1 mutations is a recently described rare disorder. The genomic variations in the fetal and maternal TPK1 gene could contribute to the variability of birth weight in normal humans. |
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TMPJ-00540
|
PEPD Protein, Human, Recombinant
PRD,Imidodipeptidase,PEPD,PeptidaseD,Prolinedipeptidase,Xaa-...
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Human
|
E. coli
|
|
PEPD belongs to the peptidase M24B family of Eukaryotic-type prolidase subfamily. PEPD is a cytosolic dipeptidase that hydrolyzes dipeptides with proline or hydroxyproline at the carboxy terminus. It is important in collagen metabolism because of the high levels of imino acids. Defects in PEPD are a cause of prolidase deficiency which is an autosomal recessive disorder associated with iminodipeptiduria. |
|
TMPJ-00504
|
Serpin A8 Protein, Human, Recombinant (His)
AGT and SERPINA8,Angiotensinogen,Serpin A8
|
Human
|
HEK293 Cells
|
|
Angiotensinogen is involved in maintaining blood pressure and in the pathogenesis of essential hypertension and preeclampsia. Genetic variations in Angiotensinogen are associated with susceptibility to essential hypertension, and can cause renal tubular dysgenesis, a severe disorder of renal tubular development. Defects in the encoding gene have also been associated with non-familial structural atrial fibrillation, and inflammatory bowel disease. |
|
TMPY-05009
|
Asp f 1 Protein, Neosartorya fumigata, Recombinant (His)
|
Neosartorya fumigata
|
HEK293 Cells
|
|
Asp f 1 (Aspergillus fumigatus allergen 1) is a major allergen produced by the mycelia of Aspergillus fumigatus. It is not present in spores and can be used as a specific marker for the detection of germination of this fungus. Allergic bronchopulmonary aspergillosis (ABPA) is an immunologically complex allergic disorder caused by the fungal pathogen Aspergillus fumigatus. |
|
TMPY-03310
|
Frizzled 4 Protein, Rat, Recombinant (hFc)
frizzled class receptor 4
|
Rat
|
HEK293 Cells
|
|
Familial exudative vitreoretinopathy (FEVR) is a hereditary blinding disorder that features defects in retinal vascular development. Mutations in FZD4 are known to cause autosomal dominant exudative vitreoretinopathy (EVR1). The mutations in FZD4 are associated with the phenotypes of retinal folds or ectopic macula in FEVR. Frizzled 4 Protein, Rat, Recombinant (hFc) is expressed in HEK293 mammalian cells with hFc tag. The predicted molecular weight is 43.2 kDa and the accession number is Q9QZH0. |
|
TMPY-00313
|
Frizzled 4 Protein, Rat, Recombinant (His)
frizzled class receptor 4
|
Rat
|
HEK293 Cells
|
|
Familial exudative vitreoretinopathy (FEVR) is a hereditary blinding disorder that features defects in retinal vascular development. Mutations in FZD4 are known to cause autosomal dominant exudative vitreoretinopathy (EVR1). The mutations in FZD4 are associated with the phenotypes of retinal folds or ectopic macula in FEVR. Frizzled 4 Protein, Rat, Recombinant (His) is expressed in HEK293 mammalian cells with His tag. The predicted molecular weight is 17.7 kDa and the accession number is A6I5Z8. |
|
TMPY-04302
|
Frizzled 4 Protein, Human, Recombinant (His)
frizzled class receptor 4,Fz-4,FEVR,hFz4,Fz4,GPCR,FzE4,EVR1,...
|
Human
|
HEK293 Cells
|
|
Familial exudative vitreoretinopathy (FEVR) is a hereditary blinding disorder that features defects in retinal vascular development. Mutations in FZD4 are known to cause autosomal dominant exudative vitreoretinopathy (EVR1). The mutations in FZD4 are associated with the phenotypes of retinal folds or ectopic macula in FEVR. Frizzled 4 Protein, Human, Recombinant (His) is expressed in HEK293 mammalian cells with His tag. The predicted molecular weight is 17.7 kDa and the accession number is Q9ULV... |
|
TMPY-00197
|
Frizzled 4 Protein, Human, Recombinant (hFc)
Fz-4,hFz4,EVR1,FEVR,Fz4,GPCR,CD344,FzE4,frizzled class recep...
|
Human
|
HEK293 Cells
|
|
Familial exudative vitreoretinopathy (FEVR) is a hereditary blinding disorder that features defects in retinal vascular development. Mutations in FZD4 are known to cause autosomal dominant exudative vitreoretinopathy (EVR1). The mutations in FZD4 are associated with the phenotypes of retinal folds or ectopic macula in FEVR. Frizzled 4 Protein, Human, Recombinant (hFc) is expressed in HEK293 mammalian cells with hFc tag. The predicted molecular weight is 43.3 kDa and the accession number is Q9ULV... |
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TMPJ-01092
|
MGAT2 Protein, Human, Recombinant (His)
Alpha-1,Mannoside Acetylglucosaminyltransferase 2,6-Mannosyl...
|
Human
|
HEK293 Cells
|
|
Mannoside Acetylglucosaminyltransferase 2 (MGAT2) is a single-pass type II membrane protein that contains the typical glycosyltransferase domains: a short N-terminal cytoplasmic domain, a hydrophobic non-cleavable signal-anchor domain and a C-terminal catalytic domain. MGAT2 catalyzes an essential step in the conversion of oligo-mannose to complex N-glycans. Defects in MGAT2 are the cause of congenital disorder of glycosylation type 2A. |
|
TMPJ-01174
|
Cystatin B Protein, Mouse, Recombinant (His)
PME,CST6cystatin B (liver thiol proteinase inhibitor)10STFBc...
|
Mouse
|
E. coli
|
|
Cystatin B, also called stefin B or liver thiol proteinase inhibitor, is a member of family 1 of the cystatin superfamily. Like Cystatin A, it is an intracellular inhibitor regulating the activities of cysteine proteases of the papain family such as cathepsins B, H and L. Defects in Cystatin-B / CSTB are the cause of progressive myoclonic epilepsy type 1 (EPM1) which is an autosomal recessive disorder characterized by severe, stimulus-sensitive myoclonus and tonic-clonic seizures. |
|
TMPH-01061
|
Cathepsin K Protein, Human, Recombinant (His)
CTSO2,Cathepsin X,Cathepsin O2,CTSK,Cathepsin O,Cathepsin K,...
|
Human
|
E. coli
|
|
Thiol protease involved in osteoclastic bone resorption and may participate partially in the disorder of bone remodeling. Displays potent endoprotease activity against fibrinogen at acid pH. May play an important role in extracellular matrix degradation. Involved in the release of thyroid hormone thyroxine (T4) by limited proteolysis of TG/thyroglobulin in the thyroid follicle lumen. Cathepsin K Protein, Human, Recombinant (His) is expressed in E. coli expression system with N-6xHis tag. The pre... |
|
TMPK-01158
|
Kallikrein 5/KLK5 Protein, Mouse, Recombinant (His)
SCTE,KLKL2,KLK5,KLK-L2,Klnc,Kallikrein c
|
Mouse
|
HEK293 Cells
|
|
The inhibition of kallikrein 5 (KLK5) has been identified as a potential strategy for treatment of the genetic skin disorder Netherton syndrome, in which loss-of-function mutations in the SPINK5 gene lead to down-regulation of the endogenous inhibitor LEKTI-1 and profound skin-barrier defects with severe allergic manifestations. To aid in the development of a medicine for this target, an X-ray crystallographic system was developed to facilitate fragment-guided chemistry and knowledge-based drug-... |
|
TMPY-02544
|
FANCA Protein, Human, Recombinant (His)
FA-H,FA1,FAA,FAH,FANCH,FACA,Fanconi anemia, complementation ...
|
Human
|
Baculovirus Insect Cells
|
|
FANCA is one of the six known Fanconi anemia gene products (FANCA, FANCC, FANCD2, FANCE, FANCF, and FANCG proteins). Fanconi anemia (FA) is a genetic disorder predisposing to aplastic anemia and cancer characterized by hypersensitivity to DNA-damaging agents and oxidative stress. FANCA associates with the IκB kinase (IKK) signalsome via interaction with IKK2. Components of the FANCA complex undergo rapid, stimulus-dependent changes in phosphorylation, which are blocked by kinase-inactive IKK2. |
|
TMPJ-01350
|
XPNPEP3 Protein, Human, Recombinant (His)
XPNPEP3,X-Pro Aminopeptidase 3,Aminopeptidase P3,APP3,Probab...
|
Human
|
E. coli
|
|
Probable Xaa-Pro Aminopeptidase 3 (XPNPEP3) is a member of the peptidase M24B family. XPNPEP3 has two isoforms and both are widely expressed. XPNPEP3 is localized in the Mitochondrion. XPNPEP3 catalyzes the release of any N-terminal amino acid, including proline, that is linked to proline, even from a dipeptide or tripeptide. Defects in XPNPEP3 are the cause of nephronophthisis-like nephropathy type 1 which is a disorder with features of nephronophthisis, a cystic kidney disease leading to end-s... |
|
TMPJ-01057
|
PSP Protein, Human, Recombinant (His)
PSPH,L-3-Phosphoserine Phosphatase,PSPase,Phosphoserine Phos...
|
Human
|
E. coli
|
|
Phosphoserine phosphatase (PSP) is an enzyme that belongs to the serB family. PSPH catalyzes magnesium-dependent hydrolysis of L-phosphoserine and is also involved in an exchange reaction between L-serine and L-phosphoserine. The reaction mechanism proceeds via the formation of a phosphoryl-enzyme intermediates. Deficiency of this protein is thought to be linked to Williams syndrome. A disorder that results in pre- and postnatal growth retardation, moderate psychomotor retardation and facial fea... |
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TMPY-03756
|
BBOX1 Protein, Human, Recombinant (His & GST)
butyrobetaine (γ), 2-oxoglutarate dioxygenase (γ-butyrobetai...
|
Human
|
Baculovirus Insect Cells
|
|
BBOX1, also known as gamma-BBH, belongs to thegamma-BBH/TMLD family. It is highly expressed in kidney and moderately expressed in liver. BBOX1 catalyzes the formation of L-carnitine from gamma-butyrobetaine, the last step in the L-carnitine biosynthetic pathway. Carnitine is essential for the transport of activated fatty acids across the mitochondrial membrane during mitochondrial beta-oxidation. BBOX1 is an inhibition target for mildronate which can be used to treatanginaandmyocardial infarctio... |
|
TMPY-04464
|
GALK1 Protein, Human, Recombinant (His & GST)
galactokinase 1,HEL-S-19,GALK,GK1
|
Human
|
Baculovirus Insect Cells
|
|
Galactokinase, also known as Galactose kinase, GALK and GALK1, is a protein which belongs to theGHMP kinase family and GalK subfamily. Galactokinase / GALK1 is a major enzyme for galactose metabolism. Galactokinase (GALK) deficiency is an autosomal recessive disorder characterized by elevation of blood galactose concentration and diminished galactose-1-phosphate, leading to the production of galactitol. Defects in GALK1 are the cause of galactosemia II ( GALCT2 ) which II is an autosomal ... |
|
TMPY-03852
|
Dopamine beta-Hydroxylase Protein, Human, Recombinant (His)
dopamine β-hydroxylase (dopamine β-monooxygenase),Dopamine β...
|
Human
|
HEK293 Cells
|
|
DBH is a 29 kDa copper-containing oxygenase. It can be detected in noradrenergic nerve terminals of the central and peripheral nervous systems, and is also expressed in chromaffin cells of the adrenal medulla. DBH contains our identical subunits, and its activity requires ascorbate as a cofactor. It functions in in the synthesis of small-molecule neurotransmitters that is membrane-bound, making norepinephrine the only transmitter synthesized inside vesicles. DBH has been shown to be associated ... |
|
TMPJ-01114
|
GAMT Protein, Human, Recombinant (His)
Guanidinoacetate N-methyltransferase,TP53I2,PIG2,GAMT
|
Human
|
E. coli
|
|
GAMT is a methyltransferase which belongs to the class I-like SAM-binding methyltransferase superfamily. It contains one RMT2 (arginine N-methyltransferase 2-like) domain and is expressed in liver. GAMT converts guanidoacetate to creatine, using S-adenosylmethionine as the methyl donor. Defects in GAMT are the cause of guanidinoacetate methyltransferase deficiency, which is an autosomal recessive disorder characterized by developmental delay/regression, mental retardation, severe disturbance of ... |
|
TMPJ-00660
|
IMPase2 Protein, Human, Recombinant (His)
Inositol-1(or 4)-Monophosphatase 2,IMPA2,IMP 2,IMPase 2,Inos...
|
Human
|
E. coli
|
|
Inositol monophosphatase 2, also known as Inositol-1(or 4)-monophosphatase 2, Myo-inositol monophosphatase A2 and IMPA2, is an enzyme which belongs to the inositol monophosphatase family. IMPA2 catalyzes the dephosphoylration of inositol monophosphate with cofactor Magnesium and Inhibited by high Li+ and restricted Mg2+ concentrations. IMPA2 plays an important role in phosphatidylinositol signaling. IMPA2 can use the myo-inositol monophosphates, scylloinositol 1,4-diphosphate, glucose-1-phosphat... |
|
TMPY-03332
|
PRTFDC1 Protein, Human, Recombinant (His)
HHGP,PRTFDC1,phosphoribosyl transferase domain containing 1
|
Human
|
E. coli
|
|
PRTFDC1 is a member of the purine/pyrimidine phosphoribosyltransferase family. It can bind GMP, IMP and alpha-D-5-phosphoribosyl 1-pyrophosphate (PRPP). The epigenetic silencing of PRTFDC1 by hypermethylation of the CpG island leads to a loss of PRTFDC1 function, which might be involved in squamous cell oral carcinogenesis. PRTFDC1 is a genetic modifier of HPRT-deficiency in the mouse and has important implications for unraveling the molecular etiology of lesch-Nyhan disease(LND). LND is a sever... |
|
TMPJ-00815
|
FBPase1 Protein, Human, Recombinant (His, E. coli)
6-Bisphosphate 1-Phosphohydrolase 1,FBP1,FBP,FBPase 1,D-Fruc...
|
Human
|
E. coli
|
|
Fructose-1,6-Bisphosphatase 1 (FBPase 1) is a member of the FBPase class 1 family. FBPase 1 is a gluconeogenesis regulatory protein, which catalyzes the hydrolysis of fructose 1,6-bisphosphate to fructose 6-phosphate and inorganic phosphate. FBPase 1 can assume an active R-state, or an inactive T-state. FBPase 1 deficiency is inherited as an autosomal recessive disorder mainly in the liver and causes life-threatening episodes of hypoglycemia and metabolic acidosis in newborn infants or young chi... |
|
TMPY-03437
|
DNAJC30 Protein, Human, Recombinant (His)
DnaJ (Hsp40) homolog, subfamily C, member 30,WBSCR18
|
Human
|
E. coli
|
|
DNAJC30 is a member of the DNAJ molecular chaperone homology domain-containing protein family. DNAJC30 gene is deleted in williams syndrome, a multisystem developmental disorder caused by the deletion of contiguous genes at 7q11.23. DNAJC30 is expressed in brain, heart, kidney, liver, lung, spleen, stomach and testis. It contains 1 J domain. DNAJC30 is located in the Williams-Beuren syndrome (WBS) critical region. WBS results from a hemizygous deletion of several genes on chromosome 7q11.23, tho... |
|
TMPY-03109
|
PHYH Protein, Human, Recombinant
PHYH1,RD,PAHX,LN1,phytanoyl-CoA 2-hydroxylase,LNAP1
|
Human
|
E. coli
|
|
PHYH belongs to the family of iron(II)-dependent oxygenases, which typically incorporate one atom of dioxygen into the substrate and one atom into the succinate carboxylate group. PHYH is expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle. It converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA. Defects in PHYH can cause Refsum disease (RD). RD is an autosomal recessive disorder characterized clinically by a tetrad of abnormalities: retinitis pigmentos... |
|
TMPY-03455
|
FGFR1OP Protein, Human, Recombinant (His & GST)
FGFR1 oncogene partner,FOP
|
Human
|
Baculovirus Insect Cells
|
|
FOP( fibroblast growth factor receptor 1 oncogene partner) is a largely hydrophilic protein postulated to be a leucine-rich protein family member. FOP contains 1 LisH domain. A t(6;8)(q27;p11) chromosomal translocation, fusing FOP gene and the fibroblast growth factor receptor 1 (FGFR1) gene, has been found in cases of myeloproliferative disorder. The resulting chimeric protein contains the N-terminal leucine-rich region of this encoded protein fused to the catalytic domain of FGFR1. FOP g... |
|
TMPJ-00763
|
ANG Protein, Human, Recombinant
RNASE5,ANG,Angiogenin,Ribonuclease 5,血管生成素,RNase 5
|
Human
|
E. coli
|
|
Angiogenin belongs to the pancreatic ribonuclease family. Angiogenin is primarily expressed in the liver. It may act as a tRNA-specific ribonuclease that abolishes protein synthesis by specifically hydrolyzing cellular tRNAs. Angiogenin is a potent stimulator of new blood vessel formation. And Angiogenin is endocytosed and translocated to the nucleus by binding to actin on the surface of endothelial cells. Angiogenic activity is regulated by interaction with RNH1 in vivo. In addition, Angiogenin... |
|
TMPJ-01343
|
MYOZ2 Protein, Human, Recombinant (His)
C4orf5,FATZ-Related Protein 2,MYOZ2,Myozenin-2,Calsarcin-1
|
Human
|
E. coli
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Myozenin 2 (MYOZ2) is a 264 amino acid protein that belongs to the myozenin family. MYOZ2 binds to Calcineurin, a phosphatase that is involved in calcium-dependent signal transduction in diverse cell types. MYOZ2 is one of the sarcomeric proteins and plays an important role in myofibrillogenesis and the modulation of calcineurin signaling. It may serve as intracellular binding proteins involved in linking Z line proteins such as alpha-actinin, gamma-filamin, TCAP/telethonin, LDB3/ZASP and plays ... |
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TMPJ-01277
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CRT2 Protein, Human, Recombinant
CALR3,Calreticulin-3,calreticulin-2,calsperin,CRT2
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Human
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E. coli
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Calreticulin-3 belongs to the calreticulin family, members of which are calcium binding chaperones localized mainly in the endoplasmic reticulum. It can be divided into a N-terminal globular domain, a proline-rich P-domain forming an elongated arm-like structure and a C-terminal acidic domain. During spermatogenesis process, Calreticulin-3 may act as a lectin-independent chaperone for specific client proteins such as ADAM3. Defects in CALR3 are the cause of familial hypertrophic cardiomyopathy t... |
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TMPJ-00280
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TNF R1 Protein, Mouse, Recombinant
Tnfrsf1a,Tumor necrosis factor receptor superfamily member 1...
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Mouse
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E. coli
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Tumor necrosis factor receptor superfamily member 1A (Tnfrsf1a) is a member of the tumor necrosis factor receptor superfamily. Tnfrsf1a is one of the major receptors for the tumor necrosis factor-alpha. It can activate the transcription factor NF-κB, mediate apoptosis, and function as a regulator of inflammation. Antiapoptotic protein BCL2-associated athanogene 4 (BAG4/SODD) and adaptor proteins TRADD and TRAF2 have been shown to interact with this receptor, and thus play regulatory roles in the... |
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TMPJ-00813
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QDPR Protein, Human, Recombinant (His)
Dihydropteridine Reductase,QDPR,DHPR,HDHPR,Quinoid Dihydropt...
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Human
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HEK293 Cells
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Dihydropteridine reductase, also known as HDHPR and Quinoid dihydropteridine reductase, QDPR and DHPR, belongs to the short-chain dehydrogenases/reductases (SDR) family. QDPR exists as a homodimer. QDPR is part of the pathway that recycles a substance called tetrahydrobiopterin, also known as BH4 and tryptophan hydroxylases. The regeneration of this substance is critical for the proper processing of several other amino acids in the body. Tetrahydrobiopterin also helps produce certain chemicals i... |
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TMPY-03985
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Adenosine Deaminase Protein, Human, Recombinant (His)
ADA1,adenosine deaminase,ADA
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Human
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Baculovirus Insect Cells
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Adenosine Desaminase (ADA) deficiency, is a purine metabolic disorder that cause severe combined immunodeficiency (SCID) due to the accumulation of toxic metabolites that primarily affects development, differentiation and function of T and B lymphocytes. Adenosine deaminase is a polymorphic enzyme that has an important role in immune functions and in the regulation of intracellular and extracellular concentrations of adenosine and adenosine receptor activity. ADA activity might be considered as... |
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TMPY-04303
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FAM20C Protein, Human, Recombinant (His)
GEF-CK,DMP4,RNS,DMP-4,family with sequence similarity 20, me...
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Human
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Baculovirus Insect Cells
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The Family with sequence similarity 20 member C (FAM20C) catalyzes the phosphorylation of secreted proteins, and participates in a variety of biological processes, including cell proliferation, migration, mineralization, and phosphate homeostasis. FAM20C is an evolutionarily reserved molecule highly expressed in mineralized tissues. Mutations in the Family with sequence similarity (FAM) 20 gene family are associated with mineralized tissue phenotypes in humans. Among these genes, FAM20A mutation... |
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TMPY-04318
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GRIK2 Protein, Human, Recombinant (hFc)
GLUR6,EAA4,MRT6,GLR6,glutamate receptor, ionotropic, kainate...
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Human
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HEK293 Cells
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GRIK2 (Glutamate Ionotropic Receptor Kainate Type Subunit 2, also known as GluR6) is a Protein Coding gene. The GRIK2 (one of the kainate receptors) gene resides in a genetic linkage region (6q21) associated with bipolar disorder (BPD). The gene coding for GRIK2 has been suggested as a candidate gene for autism based on its localization in the autism-specific region on chromosome 6q21 and the involvement of receptor protein in cognitive functions like learning and memory. GRIK2 belongs to the gl... |
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TMPY-03658
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ETHE1 Protein, Human, Recombinant (His)
YF13H12,HSCO,ethylmalonic encephalopathy 1
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Human
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E. coli
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ETHE1, also known as HSCO, is a sulfur dioxygenase that localizes within the mitochondrial matrix. ETHE1 probably plays an important role in metabolic homeostasis in mitochondria. It may also function as a nuclear-cytoplasmic shuttling protein that binds transcription factor RELA/NFKB3 in the nucleus and exports it to the cytoplasm. ETHE1 can suppresses p53-induced apoptosis by preventing nuclear localization of RELA. Mutations in ETHE1 gene result in ethylmalonic encephalopathy. Ethylmalonic en... |
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