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Cat No | BMA-2335 |
Conjugate | |
Type | 重组兔多抗 |
Source | Rabbit |
Size | 50 uL |
Application | WB; IHC; IF; FC |
Format | Liquid |
Concentration | Please refer to the vial lable for the specific concentration. |
Buffer | Supplied in PBS with 0.02% sodium azide,50% glycerol,pH7.3. |
Species | Human |
Storage | Store at -20℃. Avoid freeze / thaw cycles. |
Synonyms | AMACR;AMACRD;CBAS4;P504S;RACE;RM |
Purification | Affinity purification |
MolecularWeight | 42KDa |
Description | |
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. |