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Mouse Monoclonal P504S antibody (STJ98524)
Supplier: St John’s Laboratory Ltd.
Recommended applications: WB, IF
Recommended dilution: WB 1:1000-1:2000; IF 1:100-1:500
Recommended protocols: check protocols
Click or hover above images to see image description for P504S Monoclonal Antibody.
Check alternative names for the antibodyExpand
AMACR antibody,|2 methylacyl CoA racemase antibody|2-methylacyl-CoA racemase antibody|Alpha methylacyl CoA racemase antibody|Alpha-methylacyl-CoA racemase antibody|Amacr antibody|AMACR_HUMAN antibody|AMACRD antibody|CBAS4 antibody|EC 22.214.171.124 antibody|Macr1 antibody|RACE antibody|RM antibody|Anti-AMCR antibody (ab175280)
SCBT cat No: sc-136022|sc-81710|sc-514408|sc-514424|
P504S Monoclonal Antibody
|Catalogue No.|| |
Human, Mouse, Rat
P504S Monoclonal Antibody detects endogenous levels of P504S protein.
Purified recombinant human P504S (C-terminus) protein fragments expressed in Ecoli
|Recommended dilution|| |
WB 1:1000-1:2000; IF 1:100-1:500
P504S Antibody was tube-contained. Purified in buffer containing 0.1M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.2% sodium azide, 50% glycerol.
P504S Antibody was purified using affinity purification.
-20 Celsius degree. Avoid repeated freeze/thaw cycles.
|Alternative antibody names|| |
Alpha-methylacyl-CoA racemase antibody, 2-methylacyl-CoA racemase antibody
|Protein names|| |
Alpha-methylacyl-CoA racemase , 2-methylacyl-CoA racemase
|Protein 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. / (2S)-2-methylacyl-CoA = (2R)-2-methylacyl-CoA. / /
|Involvement in 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.
|Protein sequence and domain|| |
Belongs to the CaiB/BaiF CoA-transferase family.
|Protein cellular localization|| |
Peroxisome / Mitochondrion
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St John’s Laboratory Ltd.
|Product type|| |
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