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P504S rabbit pAb

CAT: 0855-ES20298-01Size: 50 µLDry Ice: NoHazardous: No
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CAT#:0855-ES20298-01Size:50 µL
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Background
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]
Description
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],
UniProt
Q9UHK6
Swiss Prot
Q9UHK6
Reactivity
Human; Mouse; Rat
Immunogen
Synthesized peptide derived from human P504S AA range: 271-320
Clonality
Polyclonal
Source
Rabbit
Applications
WB; ELISA
Concentration
1 mg/ml
Dilution
WB 1:1000-2000 ELISA 1:5000-20000
Storage Conditions
-20°C/1 year
Fragment
IgG
Subcellular Location
Peroxisome . Mitochondrion .
Other Product Names
Alpha-methylacyl-CoA racemase (EC 5.1.99.4;2-methylacyl-CoA racemase)
Gene ID (Human)
23600

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