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Search Results for " ataxia "

Targets Recommended: ATM/ATR

37

抑制剂 & 化合物

5

天然产物

11

重组蛋白

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Cat. No.	Product Name	Target	Signaling Pathways
T5175	azd1390	ATM/ATR	DNA Damage/DNA Repair; PI3K/Akt/mTOR signaling
	AZD1390 是一种可口服的，可渗透脑的ATM 高度选择性抑制剂，IC50为 0.78 nM。
T9989	AChE-IN-27	Others	Others
	AChE-IN-27是一种用于高通量测定的小分子。
T1821	CGK733 CGK 733	ATM/ATR	DNA Damage/DNA Repair; PI3K/Akt/mTOR signaling
	CGK733 (CGK 733) 是一种有效的选择性 ATM/ATR 抑制剂，用于癌症研究。
T6261	CP-466722 CP 466722,CP466722	ATM/ATR	DNA Damage/DNA Repair; PI3K/Akt/mTOR signaling
	CP-466722 是一种有效且可逆的 ATM 抑制剂，IC50值为 4.1 μM。它不会抑制细胞中的 ATR 和 PI3K 或 PIKK 家族成员。
T1958	AZ20	ATM/ATR; mTOR	DNA Damage/DNA Repair; PI3K/Akt/mTOR signaling
	AZ20 是一种有效且特异性的 ATR 激酶抑制剂，IC50值为 5 nM。它还抑制mTOR 活性，IC50值为 38 nM。
T4443	AZ32	ATM/ATR	DNA Damage/DNA Repair; PI3K/Akt/mTOR signaling
	AZ32 是一种口服生物利用度和血脑屏障穿透性 ATM 抑制剂，对 ATM 酶的 IC50 <6.2 nM，对细胞内 ATM 的 IC50 为 0.31 μM。
T6770	AZD0156	Apoptosis; ATM/ATR	Apoptosis; DNA Damage/DNA Repair; PI3K/Akt/mTOR signaling
	AZD0156 是一种口服活性 ATM 选择性抑制剂，IC50为 0.58 nM。它抑制 ATM 介导的信号转导，防止 DNA 损伤检查点激活，破坏 DNA 损伤修复，起抗肿瘤作用。
T3338	Ceralasertib AZD6738	ATM/ATR	DNA Damage/DNA Repair; PI3K/Akt/mTOR signaling
	Ceralasertib (AZD6738) 是一种具有口服活性的选择性 ATR 激酶抑制剂，IC50 为 1 nM。
T2084	ETP-46464	ATM/ATR; DNA-PK; PI3K; mTOR	DNA Damage/DNA Repair; PI3K/Akt/mTOR signaling
	ETP-46464 是一种有效且特异性的 ATR 抑制剂，IC50为 25 nM。
T8922	SRS11-92 AA9	Ferroptosis	Apoptosis
	SRS11-92 (AA9) 是一种铁死亡抑制剂和 Ferrostatin-1 的衍生物。它抑制 Erastin 诱导的 HT-1080 人纤维肉瘤细胞铁致细胞死亡，EC50为6 nM。
T3032	VE-821 ATR Inhibitor IV	ATM/ATR	DNA Damage/DNA Repair; PI3K/Akt/mTOR signaling
	VE-821 (ATR Inhibitor IV) 是一种有效的 ATP 竞争性的ATR 抑制剂，Ki 为 13 nM，IC50为 26 nM。
T7122	AZD-7648	DNA-PK	DNA Damage/DNA Repair; PI3K/Akt/mTOR signaling
	AZD-7648 是一种 DNA 依赖性蛋白激酶 (DNA-PK) 抑制剂，在酶试验中 IC50 为 0.63 nM，具有抗肿瘤活性。
T2685	KU-55933 ATM Kinase Inhibitor	ATM/ATR; DNA-PK; PI3K; mTOR; Autophagy	Autophagy; DNA Damage/DNA Repair; PI3K/Akt/mTOR signaling
	KU-55933 (ATM Kinase Inhibitor) 是一种 ATM 抑制剂，IC50和 Ki 值分别为 12.9 和 2.2 nM。它对 ATM 的选择性比对 PI3K/PI4K、 DNA-PK、ATR 和 mTOR 高。
T6612	NU6027	ATM/ATR; DNA-PK; CDK	Cell Cycle/Checkpoint; DNA Damage/DNA Repair; PI3K/Akt/mTOR signaling
	NU6027 是一种有效的 ATR/CDK 抑制剂，抑制 CDK1/2、ATR 和 DNA-PK，Ki 为 2.5 μM/1.3 μM、0.4 μM 和 2.2 μM。它以 ATR 依赖性方式增强羟基脲和顺铂的细胞毒性。
T9522	Rimtuzalcap CAD-1883	Potassium Channel	Membrane transporter/Ion channel
	Rimtuzalcap (CAD-1883) 是首创的，选择性的小电导钙激活钾通道 (SK 通道) 正变构调节剂。Rimtuzalcap 可用于运动障碍的研究，包括脊髓小脑性共济失调 (SCA)和特发性震颤 (ET)。
T7318	Elimusertib BAY-1895344	ATM/ATR	DNA Damage/DNA Repair; PI3K/Akt/mTOR signaling
	Elimusertib (BAY-1895344) 是一种可口服的选择性ATR 抑制剂，IC50值为 7 nM。它具有抗肿瘤活性，可研究实体瘤和淋巴瘤。
T19663	Triheptanoin UX 007,IND106011,三庚酸甘油脂,UX007,UX-007,IND 106011,IND-106011	Others	Others
	Triheptanoin (IND 106011) 是合成的中链甘油三酯 (MCT)，由甘油主链上的三个奇数链 7-碳(庚酸)脂肪酸组成，可以用于研究遗传代谢紊乱。
T17174	Troriluzole BHV4157,BHV-4157,BHV-4157a,FC 4157,BHV 4157	transporter	Metabolism
	Troriluzole 是一种具有口服活性谷氨酸调节剂，具有抗癌活性。Troriluzole 可提高位于神经胶质细胞的兴奋性氨基酸转运体的表达，可用于研究脊髓小脑性共济失调、阿尔茨海默病和广泛性焦虑症 (GAD) 。
T16345	NS13001 N-(4-chlorophenyl)-2-(3,5-dimethylpyrazol-1-yl)-9-methylpurin-6-amine,N-(4-Chlorophenyl)-2-(3,5-dimethyl-1H-pyrazol-1-yl)-9-methyl-9H-purin-6-amine	Potassium Channel	Membrane transporter/Ion channel
	NS13001 (N-(4-Chlorophenyl)-2-(3,5-dimethyl-1H-pyrazol-1-yl)-9-methyl-9H-purin-6-amine) 是口服有效的 SK 通道选择性正向变构调节剂。NS13001 对 SK2 和 SK3 通道的 EC50分别为 1.8 μM 和 0.14 μM。NS13001 在脊髓小脑共济失调 2 型和其他小脑共济失调方面具有研究价值。
T10406	Tuvusertib M1774,ATR inhibitor 1	Apoptosis; ATM/ATR; Others; Chk	Apoptosis; Cell Cycle/Checkpoint; DNA Damage/DNA Repair; Others; PI3K/Akt/mTOR signaling
	Tuvusertib (M1774) 是一种可口服的共济失调毛细血管扩张和 Rad3相关(ATR)激酶抑制剂(Ki< 1 µΜ)，具有选择性和潜在的抗肿瘤活性。Tuvusertib 选择性抑制 ATR 活性，阻断丝氨酸/苏氨酸蛋白激酶检查点激酶1 (CHK1)的下游磷酸化，从而抑制 DNA 损伤检查点激活，破坏 DNA 损伤修复，诱导肿瘤细胞凋亡。
T20826	Dioxabenzofos Salithion	Others	Others
	Dioxabenzofos, a pesticide, could cause reversible ataxia in mice, chicken, and sheep.
T78711	A011	ATM/ATR	DNA Damage/DNA Repair; PI3K/Akt/mTOR signaling
	A011，作为一种选择性的ataxia-telangiectasia mutated (ATM)抑制剂，其IC50为1.0 nM，能够在与CPT-11联合应用时，诱导Apoptosis及G2/M期细胞周期阻滞，显示出显著的抗肿瘤活性。
T76419	Protein Kinase C γ Peptide
	Protein Kinase C γ Peptide，Protein Kinase Cγ 的肽片段，为显性共济失调的致病蛋白，能够负向调节隐性共济失调相关失联蛋白的核输入。
T39523	Antitumor agent-28	Others	Others
	Antitumor agent-28 is a compound that specifically targets and inhibits the activity of the ataxia telangiectasia mutated (ATM) kinase. By doing so, it effectively impedes the progression of ATM-mediated diseases and demonstrates significant anti-cancer activity.
T69017	Ibotenic acid hydrate	Others	Others
	Ibotenic acid hydrate is a neurotoxic isoxazole (similar to Kainic acid and Muscimol) found in Amanita mushrooms. It causes motor depression, ataxia, and changes in mood, perceptions and feelings, and is a potent excitatory amino acid agonist.
T40212	ATR-IN-4	Others	Others
	ATR-IN-4, a potent inhibitor of ATR (Ataxia telangiectasia mutated gene Rad 3-associated kinase), restricts the growth of DU145 prostate cancer cells and NCI-H460 lung cancer cells, with IC 50 values of 130.9 nM and 41.33 nM, respectively.
T40077	ATR-IN-9	Others	Others
	ATR-IN-9 is a highly effective inhibitor of Ataxia-telangiectasia and RAD-3-related protein kinase (ATR). With an IC50 value as low as 10 nM, ATR-IN-9 demonstrates exceptional potency.
T83753	RAD17-derived Peptide TFA
	RAD17衍生肽是一种针对共济失调-毛细血管扩张及RAD3相关蛋白/激酶(ATR)的肽类底物。它已被用于识别ATR的抑制剂。
T26981	Ceralasertib formate Ceralasertib,AZD-6738,AZD 6738,AZD6738	Others	Others
	Ceralasertib is an orally available inhibitor of ataxia telangiectasia and rad3 related (ATR) kinase. Ceralasertib selectively inhibits ATR activity by blocking the downstream phosphorylation of the serine/threonine protein kinase CHK1. This prevents ATR-
T81638	NSC363998 free base	Others	Others
	NSC363998 (free base) 是一种具口服活性的化合物，能够抑制 rCGG90 诱导的神经毒性，并可应用于脆性 X 相关震颤/共济失调综合征 (FXTAS) 等神经退行性疾病的研究。
T62965	TDP1 Inhibitor-2	Others	Others
	TDP1 Inhibitor-2 是一种 TDP1 (酪氨酰 -DNA 磷酸二酯酶 1) 的有效抑制剂 (IC50: 99 nM)。TDP1 Inhibitor-2 对 SCAN1 (脊髓小脑共济失调综合征伴轴突神经病变) 具有抑制作用 (IC50: 3.5 μM)。
T83950	ARN 11391	Others	Others
	ARN 11391是ITPR1 (Inositol 1,4,5-trisphosphate (IP3) receptor type 1)的增效剂，它直接作用于ITPR1，从而增强细胞内钙离子信号。ARN 11391还能增强表达突变型ITPR1细胞中的钙信号，这与SCA29型脊髓小脑共济失调有关。
T36003	1(R)-(Trifluoromethyl)oleyl alcohol	Others	Others
	1(R)-(Trifluoromethyl)oleyl alcohol is an analog of oleic acid .1It inhibits ferroptosis induced by erastin in primary fibroblasts isolated from patients with Friedreich ataxia, a neuro- and cardiodegenerative disorder characterized by loss or impaired activity of frataxin (FXN), when used at concentrations of 5, 10, or 20 μM. 1(R)-(Trifluoromethyl)oleyl alcohol (5 μM) reduces lipid peroxidation induced byFXNsiRNA knockdown in NBT human myoblasts. 1.Cotticelli, M.G., Forestieri, R., Xia, S., et ...
T62563	ATR-IN-12	Others	Others
	ATR-IN-12 (Compound 5g) 是一种共济失调毛细血管扩张和 Rad3 相关 (ATR) 激酶的有效抑制剂 (IC50: 0.007 μM)。ATR-IN-12 能够明显降低 ATR 及其下游信号蛋白的磷酸化水平，具有良好的抗肿瘤效果。ATR-IN-12 是一种极有潜力的先导化合物，能够用于后续对 ATR 激酶的药物发现的研究。
T62651	ATR-IN-11	Others	Others
	ATR-IN-11 (Compound Hit01) 是一种共济失调毛细血管扩张症和 Rad3 相关 (ATR) 激酶的有效抑制剂。ATR 激酶是 DNA 损伤反应 (DDR) 中的一个关键调节蛋白，负责感知复制压力 (RS)。ATR-IN-11 是一种有希望的先导化合物，能够用于发现后续针对 ATR 激酶的药物。 ATR-IN-11具有潜力进行癌症疾病的研究。
T65396	Piperazine 2HCl	Others	Others
	Piperazine (2HCl) is gamma-aminobutyric acid (GABA) agonists and its major effects appear to be on the central nervous system. Piperazine was the anthelmintic with the greatest number of reports of toxicoses and suspected toxicoses in cats. Piperazine neurotoxicity in cats and dogs usually was manifested by muscle tremors, ataxia, and/or behavioral disturbances within 24 hours after estimated daily dose(s) between 20 and 110 mg/kg[1]. For di-substituted derivatives, ciprofloxacin was selected an...
T35942	AZ 5704	Others	Others
	Potent and selective ATM kinase inhibitor (IC50 = 0.6 nM in an enzyme inhibition assay). Exhibits > 600-fold selectivity for ATM over other kinases. Inhibits ATM kinase in an in vitro cellular assay (IC50 = 0.33 μM). Potentiates the antitumor effects of the topoisomerase 1 inhibitor irinotecan in tumor bearing, immunocompromised mice. Orally bioavailable. Degorce et al (2016) Discovery of novel 3-quinoline carboxamides as potent, selective and orally bioavailable inhibitors of ataxia telangiecta...

化合物

Cat.No: T5175

Target: ATM/ATR

Cat.No: T9989

Target: Others

Cat.No: T1821

Synonym: CGK 733

Target: ATM/ATR

Cat.No: T6261

Synonym: CP 466722,CP466722

Target: ATM/ATR

Cat.No: T1958

Target: ATM/ATR, mTOR

Cat.No: T4443

Target: ATM/ATR

Cat.No: T6770

Target: Apoptosis, ATM/ATR

Cat.No: T3338

Synonym: AZD6738

Target: ATM/ATR

Cat.No: T2084

Target: ATM/ATR, DNA-PK, PI3K, mTOR

Cat.No: T8922

Synonym: AA9

Target: Ferroptosis

Cat.No: T3032

Synonym: ATR Inhibitor IV

Target: ATM/ATR

Cat.No: T7122

Target: DNA-PK

Cat.No: T2685

Synonym: ATM Kinase Inhibitor

Target: ATM/ATR, DNA-PK, PI3K, mTOR, Autophagy

Cat.No: T6612

Target: ATM/ATR, DNA-PK, CDK

Cat.No: T9522

Synonym: CAD-1883

Target: Potassium Channel

Cat.No: T7318

Synonym: BAY-1895344

Target: ATM/ATR

Cat.No: T19663

Synonym: UX 007,IND106011,三庚酸甘油脂,UX007,UX-007,IND 106011,IND-106011

Target: Others

Cat.No: T17174

Synonym: BHV4157,BHV-4157,BHV-4157a,FC 4157,BHV 4157

Target: transporter

Cat.No: T16345

Synonym: N-(4-chlorophenyl)-2-(3,5-dimethylpyrazol-1-yl)-9-methylpurin-6-amine,N-(4-Chlorophenyl)-2-(3,5-dimethyl-1H-pyrazol-1-yl)-9-methyl-9H-purin-6-amine

Target: Potassium Channel

Cat.No: T10406

Synonym: M1774,ATR inhibitor 1

Target: Apoptosis, ATM/ATR, Others, Chk

Cat.No: T20826

Synonym: Salithion

Target: Others

Cat.No: T78711

Target: ATM/ATR

Protein Kinase C γ Peptide

Cat.No: T76419

Antitumor agent-28

Cat.No: T39523

Target: Others

Ibotenic acid hydrate

Cat.No: T69017

Target: Others

Cat.No: T40212

Target: Others

Cat.No: T40077

Target: Others

RAD17-derived Peptide TFA

Cat.No: T83753

Ceralasertib formate

Cat.No: T26981

Synonym: Ceralasertib,AZD-6738,AZD 6738,AZD6738

Target: Others

NSC363998 free base

Cat.No: T81638

Target: Others

TDP1 Inhibitor-2

Cat.No: T62965

Target: Others

Cat.No: T83950

Target: Others

1(R)-(Trifluoromethyl)oleyl alcohol

Cat.No: T36003

Target: Others

Cat.No: T62563

Target: Others

Cat.No: T62651

Target: Others

Piperazine 2HCl

Cat.No: T65396

Target: Others

Cat.No: T35942

Target: Others

Cat. No.	Product Name	Target	Signaling Pathways
TN1673	Garcinone C 伽升沃 C,伽升沃C	ATM/ATR; CDK; STAT; AChR; Antifection	Cell Cycle/Checkpoint; DNA Damage/DNA Repair; JAK/STAT signaling; Microbiology/Virology; Neuroscience; PI3K/Akt/mTOR signaling; Stem Cells
	Garcinone C 是一种从 Garcinia oblongifolia Champ 中提取的黄酮衍生物，具有抗炎、收敛和促进肉芽的活性。 Garcinone C 是一种 AChE 抑制剂，对某些癌症具有潜在的细胞毒性作用。
T3392	Sinapine thiocyanate	P-gp; AChE	Membrane transporter/Ion channel; Neuroscience
	Sinapine thiocyanate 是萝卜中分离出来的一种生物碱，具有抗炎、抗氧化、抗肿瘤、抗血管生成和放射防护作用。它还是乙酰胆碱酯酶抑制剂，可用于研究阿尔茨海默症，共济失调，重症肌无力和帕金森氏病。
T2S1200	Sinapine	Antioxidant; P-gp; AChE	Membrane transporter/Ion channel; Neuroscience; oxidation-reduction
	Sinapine 是萝卜中分离出来的一种生物碱，具有抗炎、抗氧化、抗肿瘤、抗血管生成和放射防护作用。它还是乙酰胆碱酯酶抑制剂，可用于研究阿尔茨海默症，共济失调，重症肌无力和帕金森氏病。
T10933	D-Cysteine	Endogenous Metabolite; Antibacterial	Metabolism; Microbiology/Virology
	D-Cysteine 是一种大肠杆菌生长的有力抑制剂，是半胱氨酸的 D 型异构体。它由 D-氨基酸氧化酶介导产生 H2S，是一种对抗小脑共济失调的神经保护剂。 D-半胱氨酸可以抑制由变形链球菌和链球菌血红蛋白形成的两种生物膜的生长和致龋性。
T38606	Sinapine hydroxide	Others	Others
	Sinapine hydroxide, an alkaloid derived from the seeds of cruciferous plants, demonstrates a variety of beneficial properties including anti-inflammatory, anti-oxidant, anti-tumor, anti-angiogenic, and radio-protective effects. Additionally, it acts as an inhibitor of acetylcholinesterase (AChE), making it valuable for researching neurodegenerative conditions such as Alzheimer’s disease, ataxia, myasthenia gravis, and Parkinson’s disease[4].

天然产物

Cat.No: TN1673

Synonym: 伽升沃 C,伽升沃C

Target: ATM/ATR, CDK, STAT, AChR, Antifection

Sinapine thiocyanate

Cat.No: T3392

Target: P-gp, AChE

Cat.No: T2S1200

Target: Antioxidant, P-gp, AChE

Cat.No: T10933

Target: Endogenous Metabolite, Antibacterial

Sinapine hydroxide

Cat.No: T38606

Target: Others

Species：

Expression System：

Tag：

Cat. No.	Product Name	Species	Expression System
TMPH-01356	Frataxin/FXN Protein, Human, Recombinant (His & Myc) Frataxin, mitochondrial,Friedreich ataxia protein,F...	Human	E. coli
	Frataxin/FXN Protein, Human, Recombinant (His & Myc) is expressed in E. coli expression system with N-10xHis and C-Myc tag. The predicted molecular weight is 28.1 kDa and the accession number is Q16595.
TMPH-00963	Ataxin-2 Protein, Human, Recombinant (His) TNRC13,ATX2,ATXN2,Ataxin-2,Trinucleotide repeat-containing g...	Human	E. coli
	Involved in EGFR trafficking, acting as negative regulator of endocytic EGFR internalization at the plasma membrane. Ataxin-2 Protein, Human, Recombinant (His) is expressed in E. coli expression system with N-6xHis tag. The predicted molecular weight is 37.0 kDa and the accession number is Q99700.
TMPY-02953	TEM8/ANTXR1 Protein, Human, Recombinant (hFc) ATR,TEM8,anthrax toxin receptor 1,GAPO	Human	HEK293 Cells
	ANTXR1 contains 1 VWFA domain and belongs to the ATR family. ATR (Ataxia telangiectasia and Rad3 related) and ATM (Ataxia telangiectasia mutated) are closely related kinases that are activated by DNA damage. They are serine-threonine protein kinases and belongs to the phosphatidylinositol 3' kinase-like kinase (PIKK) family. Upon recruitment by the DNA damage binding proteins/complexes (ATRIP for ATR; MRN for ATM), ATM/ATR initiate the DNA damage checkpoint by phosphorylating a number of key pro...
TMPY-01085	VLDLR Protein, Human, Recombinant (His) VLDLRCH,CAMRQ1,CHRMQ1,very low density lipoprotein receptor,...	Human	HEK293 Cells
	The very low density lipoprotein receptor, known as VLDLR, is a single-pass type 1 integral membrance protein and a member of the LDL receptor family. This receptor family includes LDL receptor, LRP, megalin, VLDLR and ApoER2, and is characterized by a cluster of cysteine-rich class A repeats, epidermal growth factor (EGF)-like repeats, YWTD repeats and an O-linked sugar sdomain. VLDLR contains 3 EGF-like domains, 8 LDL-receptor class A domains, as well as 6 LDL-receptor class B repeats, and is ...
TMPK-00015	Cpn10/HSPE1 Protein, Human/Mouse/Goat, Recombinant (His & Avi) GROES,Hsp10,Chaperonin 10,HSPE1,EPF,CPN10	Human,Mouse,Goat	E. coli
	Cpn10/HSPE, a 10-kDa heat shock protein, is a novel interacting partner of NPAT. A pool of Cpn10 is colocalized with NPAT foci during G1 and S phases in nuclei.Cpn10 is important for S phase progression and cell proliferation. Interaction of Heat Shock Protein Cpn10 with the Cyclin E/Cdk2 Substrate Nuclear Protein Ataxia-Telangiectasia (NPAT) Is Involved in Regulating Histone Transcription. Cpn10/HSPE1 Protein, Human/Mouse/Goat, Recombinant (His & Avi) is expressed in E. coli expression system w...
TMPK-00016	Cpn10/HSPE1 Protein, Human/Mouse/Goat, Recombinant (His & Avi), Biotinylated Hsp10,Chaperonin 10,HSPE1,GROES,EPF,Cpn10	Human,Mouse,Goat	E. coli
	Cpn10/HSPE, a 10-kDa heat shock protein, is a novel interacting partner of NPAT. A pool of Cpn10 is colocalized with NPAT foci during G1 and S phases in nuclei.Cpn10 is important for S phase progression and cell proliferation. Interaction of Heat Shock Protein Cpn10 with the Cyclin E/Cdk2 Substrate Nuclear Protein Ataxia-Telangiectasia (NPAT) Is Involved in Regulating Histone Transcription. Cpn10/HSPE1 Protein, Human/Mouse/Goat, Recombinant (His & Avi), Biotinylated is expressed in E. coli expre...
TMPY-00320	APOA1BP Protein, Human, Recombinant (His) YJEFN1,apolipoprotein A-I binding protein,AIBP	Human	HEK293 Cells
	APOA1BP, now renamed NAXE, encodes an epimerase essential in the cellular metabolite repair for NADHX and NADPHX. The enzyme catalyzes the epimerization of NAD(P)HX, thereby avoiding the accumulation of toxic metabolites.Pathogenic biallelic mutations in NAXE in children from four families with (sub-) acute-onset ataxia, cerebellar edema, spinal myelopathy, and skin lesions.
TMPK-00928	VLDLR Protein, Mouse, Recombinant (His) CHRMQ1,VLDLR,FLJ35024,VLDLRCH,RP11-320E16.1,VLDL-R	Mouse	HEK293 Cells
	VLDLR cerebellar hypoplasia (VLDLR-CH) is characterized by non-progressive congenital ataxia that is predominantly truncal and results in delayed ambulation, moderate-to-profound intellectual disability, dysarthria, strabismus, and seizures.VLDLR-CH is inherited in an autosomal recessive manner. Carrier testing for at-risk relatives, prenatal testing for a pregnancy at increased risk and preimplantation genetic testing are possible when the pathogenic variants in a family are known.
TMPY-04476	MVK Protein, Human, Recombinant (His & GST) POROK3,LRBP,MVLK,MK,mevalonate kinase	Human	Baculovirus Insect Cells
	Mevalonate kinase belongs to the GHMP kinase family, Mevalonate kinase subfamily. It can be found in a wide variety of organisms from bacteria to mammals. Mevalonate kinase may be a regulatory site in the cholesterol biosynthetic pathway. Defects in mevalonate kinase can cause mevalonic aciduria (MEVA). It is an accumulation of mevalonic acid which causes a variety of symptoms such as psychomotor retardation, dysmorphic features, cataracts, hepatosplenomegaly, lymphadenopathy, anemia, hypotonia,...
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...
TMPJ-01087	TIM14 Protein, S. cerevisiae, Recombinant Mitochondrial import inner membrane translocase subunit TIM1...	S. cerevisiae	E. coli
	Mitochondrial import inner membrane translocase subunit TIM14 (TIM14) is an essential component of the PAM complex. PAM complex is required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. In the complex, TIM14 is required to stimulate activity of mtHSP70 (SSC1). TIM14 belongs to the DnaJ family, which has been involved in Hsp40/Hsp70 chaperone systems. As a mitochondrial chaperone, TIM14 functions as p...

重组蛋白

Frataxin/FXN Protein, Human, Recombinant (His & Myc)

Cat.No: TMPH-01356

Species: Human

Expression System: E. coli

Ataxin-2 Protein, Human, Recombinant (His)

Cat.No: TMPH-00963

Species: Human

Expression System: E. coli

TEM8/ANTXR1 Protein, Human, Recombinant (hFc)

Cat.No: TMPY-02953

Species: Human

Expression System: HEK293 Cells

VLDLR Protein, Human, Recombinant (His)

Cat.No: TMPY-01085

Species: Human

Expression System: HEK293 Cells

Cpn10/HSPE1 Protein, Human/Mouse/Goat, Recombinant (His & Avi)

Cat.No: TMPK-00015

Species: Human,Mouse,Goat

Expression System: E. coli

Cpn10/HSPE1 Protein, Human/Mouse/Goat, Recombinant (His & Avi), Biotinylated

Cat.No: TMPK-00016

Species: Human,Mouse,Goat

Expression System: E. coli

APOA1BP Protein, Human, Recombinant (His)

Cat.No: TMPY-00320

Species: Human

Expression System: HEK293 Cells

VLDLR Protein, Mouse, Recombinant (His)

Cat.No: TMPK-00928

Species: Mouse

Expression System: HEK293 Cells

MVK Protein, Human, Recombinant (His & GST)

Cat.No: TMPY-04476

Species: Human

Expression System: Baculovirus Insect Cells

PHYH Protein, Human, Recombinant

Cat.No: TMPY-03109

Species: Human

Expression System: E. coli

TIM14 Protein, S. cerevisiae, Recombinant

Cat.No: TMPJ-01087

Species: S. cerevisiae

Expression System: E. coli

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