货号 |
bsm-33053M-1 |
品牌 |
|
浓度 |
|
货期 |
现货 |
英文名称 |
Mouse Anti-AMACR antibody |
中文名称 |
Mouse Anti-AMACR antibody |
研究领域 |
肿瘤,细胞生物,免疫学,信号转导,新陈代谢,表观遗传学, |
英文别名 |
Alpha-methylacyl-CoA racemase; 2-methylacyl-CoA racemase; RM; RACE; CBAS4; P504S; AMACRD; AMACR_HUMAN; |
反应物种(已验证) |
Human,Mouse |
反应物种(预测) |
Rat |
产品应用(已验证) |
WB,IHC |
推荐稀释比例 |
WB=1:500-1000,IHC-P=1:100-500, |
克隆类型 |
单克隆 |
克隆号 |
6B3 |
抗体来源 |
Mouse |
理论分子量 |
42 |
细胞定位 |
细胞浆 |
性状 |
Liquid |
免疫原 |
Recombinant human AMACR protein |
抗原表位 |
full length |
亚型 |
IgG |
纯化方法 |
affinity purified by Protein G |
SUBCELLULAR |
Peroxisome. Mitochondrion. |
SIMILARITY |
Belongs to the CaiB/BaiF CoA-transferase family. |
Function |
Racemization of 2-methyl-branched fatty acid CoA esters. Responsible for the conversion of pristanoyl-CoA and C27-bile acyl-CoAs to their (S)-stereoisomers. |
DISEASE |
Alpha-methylacyl-CoA racemase deficiency (AMACRD) [MIM:614307]: A rare autosomal recessive peroxisomal disorder characterized by elevated plasma concentrations of pristanic acid C27-bile-acid intermediates, and adult onset of variable neurodegenerative sy |
SWISS |
Q9UHK6 |
Gene ID |
23600 |
保存条件 |
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
Important Note |
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
英文介绍 |
This gene encodes a racemase. The encoded enzyme interconverts pristanoyl-CoA and C27-bile acylCoAs between their (R)- and (S)-stereoisomers. The conversion to the (S)-stereoisomers is necessary for degradation of these substrates by peroxisomal beta-oxidation. Encoded proteins from this locus localize to both mitochondria and peroxisomes. Mutations in this gene may be associated with adult-onset sensorimotor neuropathy, pigmentary retinopathy, and adrenomyeloneuropathy due to defects in bile acid synthesis. Alternatively spliced transcript variants have been described. Read-through transcription also exists between this gene and the upstream neighboring C1QTNF3 (C1q and tumor necrosis factor related protein 3) gene. [provided by RefSeq, Mar 2011] |