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AMACR Rabbit pAb (bs-0840R)  
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產品編號 bs-0840R
英文名稱 AMACR Rabbit pAb
中文名稱 α-甲基酰基輔酶A消旋酶抗體
別    名 AMACR_HUMAN; alpha-methylacyl-CoA racemase; EC:5.1.99.4; 2-methylacyl-CoA racemase; RM; RACE; CBAS4; P504S; AMACRD;  
研究領域 腫瘤  細胞生物  免疫學  染色質和核信號  信號轉導  新陳代謝  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human,Mouse,Rat
產品應用 WB=1:500-2000,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 42 kDa
檢測分子量
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 Recombinant Mouse AMACR protein 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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]

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.

Subcellular Location:
Peroxisome. Mitochondrion.

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 symptoms affecting the central and peripheral nervous systems. Features may include seizures, visual failure, sensorimotor neuropathy, spasticity, migraine, and white matter hyperintensities on brain imaging. Note=The disease is caused by mutations affecting the gene represented in this entry.
Congenital bile acid synthesis defect 4 (CBAS4) [MIM:214950]: A disorder characterized by the presence of trihydroxycoprostanic acid in the bile and absence of cholic acid. Patients manifest neonatal jaundice, intrahepatic cholestasis and bile duct deficiency. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the CaiB/BaiF CoA-transferase family.

Database links:

Entrez Gene: 23600 Human

Omim: 604489 Human

SwissProt: Q9UHK6 Human

Unigene: 508343 Human



AMACR的優點在于它是癌癥特異性,在癌癥組織中高表達。 AMACR亦可用作其他癌癥的診斷標志物。對各種癌癥細胞進行檢查后發現,結腸直腸癌、卵巢癌、乳腺癌、膀胱癌、肺癌、淋巴瘤和黑素瘤都過度表達AMACR,以結腸直腸癌和前列腺癌表達最高。
AMACR是一種新型前列腺癌標記物,在前列腺癌中胞漿表達較多,正常表到較少.
產品圖片
Sample: Lane 1: Mouse Liver tissue lysates Lane 2: Mouse Kidney tissue lysates Lane 3: Rat Liver tissue lysates Lane 4: Rat Kidney tissue lysates Primary: Anti-AMACR (bs-0840R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 42 kDa Observed band size: 38 kDa
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