AMACRacemase RMab | MyBioSource MBS370005 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

AMACRacemase RMab

catalog :

MBS370005

quantity :

3 ml

price :

255 USD

clonality :

monoclonal

host :

rabbit

conjugate :

nonconjugated

clone name :

13H4

reactivity :

human

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS370005

products type :

Antibody

products full name :

AMACRacemase RMab

products short name :

AMACRacemase

products name syn :

AMACRacemase RMab

other names :

Alpha-methylacyl-CoA racemase; Alpha-methylacyl-CoA racemase; alpha-methylacyl-CoA racemase; OTTHUMP00000115916; OTTHUMP00000115917; OTTHUMP00000219978; OTTHUMP00000219979; OTTHUMP00000219980; 2-methylacyl-CoA racemase; alpha-methylacyl-CoA racemase; 2-methylacyl-CoA racemase

products gene name :

AMACRacemase

other gene names :

AMACR; AMACR; RM; RACE; CBAS4

uniprot entry name :

AMACR_HUMAN

clonality :

Monoclonal

isotype :

IgG

clone :

13H4

host :

Rabbit

form :

P504S/AMACR is a rabbit monoclonal antibody derived from cell culture supernatant that is concentrated, dialyzed, filter sterilized and diluted in buffer pH 7.5, containing BSA and sodium azide as a preservative.

storage stability :

Store at 2 to 8 degree C in the dark.

other info1 :

Control: Prostatic Adenocarcinoma. Localization: Cytoplasmic

other info2 :

Reactivity Note: Paraffin, Frozen

products description :

AMACR (P504S) is an acronym for the protein alpha-methylacyl CoA racemase that helps to metabolize certain fatty acids within the body. AMACR has been recently described as a prostate cancer-specifi c gene that encodes a protein involved in the beta-oxidation of branched chain fatty acids. Expression of AMACR protein is found in Prostatic Adenocarcinoma but not in benign prostatic tissue. It stains premalignant lesions of the prostate: High-Grade Prostatic Intraepithelial Neoplasia (PIN) and Atypical Adenomatous Hyperplasia. Several studies have suggested that AMACR can be used as a prostate cancer biomarker.High expression of AMACR (P504S) protein is usually found in Prostatic Adenocarcinoma but not in benign prostatic tissue by immunohistochemical staining in paraffin-embedded tissues. Using AMACR as a positive marker along with basal-cell staining (34betaE12 or p63) as a negative marker could help to confirm the diagnosis of small foci of Prostate Carcinoma on needle biopsies.

ncbi gi num :

13626118

ncbi acc num :

Q9UHK6

ncbi gb acc num :

Q9UHK6

uniprot acc num :

Q9UHK6

ncbi mol weight :

42,387 Da

ncbi pathways :

Beta-oxidation Of Pristanoyl-CoA Pathway 106138!!Bile Acid And Bile Salt Metabolism Pathway 106144!!Metabolic Pathways 132956!!Metabolism Of Lipids And Lipoproteins Pathway 160976!!Peroxisomal Lipid Metabolism Pathway 106136!!Peroxisome Pathway 131226!!Peroxisome Pathway 131126!!Primary Bile Acid Biosynthesis Pathway 82938!!Primary Bile Acid Biosynthesis Pathway 299!!Synthesis Of Bile Acids And Bile Salts Pathway 106145

ncbi summary :

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]

uniprot summary :

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. Catalytic activity: (2S)-2-methylacyl-CoA = (2R)-2-methylacyl-CoA. Pathway: Lipid metabolism; bile acid biosynthesis.Lipid metabolism; fatty acid metabolism. Subcellular location: Peroxisome. Mitochondrion Ref.1. Involvement in disease: Defects in AMACR are the cause of alpha-methylacyl-CoA racemase deficiency (AMACRD) [. MIM:604489]. AMACRD results in elevated plasma concentrations of pristanic acid C27-bile-acid intermediates. It can be associated with polyneuropathy, retinitis pigmentosa, epilepsy. Ref.2Defects in AMACR are the cause of congenital bile acid synthesis defect type 4 (CBAS4) [. MIM:214950]; also known as cholestasis, intrahepatic, with defective conversion of trihydroxycoprostanic acid to cholic acid or trihydroxycoprostanic acid in bile. Clinical features include neonatal jaundice, intrahepatic cholestasis, bile duct deficiency and absence of cholic acid from bile. Ref.2 Ref.11. Sequence similarities: Belongs to the CaiB/BaiF CoA-transferase family. Sequence caution: The sequence ACL67853.1 differs from that shown. Reason: Aberrant splicing.The sequence ACL67854.1 differs from that shown. Reason: Aberrant splicing.The sequence CAB44062.1 differs from that shown. Reason: Frameshift at positions 62, 65 and 114.

size :

3 ml

price :

255 USD

more info or order :

MyBioSource product webpage