PMP22 Recombinant Rabbit Monoclonal Antibody [JM52-30]

Function

Growth arrest-specific protein 3 (GAS-3), also called peripheral myelin protein 22 (PMP22), is a protein which in humans is encoded by the PMP22 gene. PMP22 is a 22 kDa transmembrane glycoprotein made up of 160 amino acids, and is mainly expressed in the Schwann cells of the peripheral nervous system. Schwann cells show high expression of PMP22, where it can constitute 2-5% of total protein content in compact myelin. Compact myelin is the bulk of the peripheral neuron's myelin sheath, a protective fatty layer that provides electrical insulation for the neuronal axon. The level of PMP22 expression is relatively low in the central nervous system of adults. Like other membrane proteins, newly translated PMP22 protein is temporarily sequestered to the endoplasmic reticulum (ER) and Golgi apparatus for post-translational modifications. PMP22 protein is glycosylated with an N terminus-linked sugar and co-localized with the chaperone protein calnexin in the ER. After the protein is transported to the Golgi apparatus it can then become incorporated in the plasma membrane of the cell.

Background References

1. Patzig J et al. Quantitative and integrative proteome analysis of peripheral nerve myelin identifies novel myelin proteins and candidate neuropathy loci. J Neurosci 31:16369-86 (2011).

2. Koeppen AH et al. The dorsal root ganglion in Friedreich\'s ataxia. Acta Neuropathol 118:763-76 (2009).

Sequence Similarity

Belongs to the PMP-22/EMP/MP20 family.

Subcellular Location

Cell membrane.

UNIPROT

SwissProt: Q01453 Human

SwissProt: P16646 Mouse

SwissProt: P25094 Rat

Synonyms