PIp1, ID (Proteolipid protein1)

Size

£672.00

SKU:: 031197
Purity:: Purified by Euglobin precipitation.
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Mouse
Immunogen:: KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 248-277 in the central region of human Pip1, UniProt Accession #P60203. Species sequence homology: bovine, chicken, monkey, mouse, porcine, rabbit
Clone:: 538CT16.5.4
Format:: Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.
Extra Details:: This is the major myelin protein from the central nervous system. It plays an important role in the formation or maintenance of the multilamellar structure of myelin. Proteolipid protein (Plp) gene mutation in rodents causes severe CNS dysmyelination, early death, and lethal hypoxic ventilatory depression (Miller et al. 2004). Myelin proteolipid protein (PLP) is the major protein in CNS myelin, accounting for 50% of myelin-associated proteins in the central nervous system. Mutations in the Plp gene, which are X-linked, have been found in rodents, such as the myelin deficient (MD) rat, and the myelin synthesis deficient mouse (Plpmsd). These mutations cause severe dysmyelination in the CNS, associated with oligodendrocyte death and non-myelinated or poorly myelinated axons, as well as abnormal axoglial junctions (Dentinger et al. 1982; Rosenbluth 1987). Most rodents with mutations in the Plp gene develop tremors, ataxia, and seizures in the second week of life, and early death by postnatal day (p) 21–23. (Knapp 1996; Garbern et al. 1999; Yool et al. 2000). Plp mutations in humans are associated with a leukoencephalopathy, Pelizaeus-Merzbacher disease, which bears many pathophysiologic similarities to the rodents with Plp mutations (Boulloche and Aicardi 1986). The mechanisms by which mutations in the Plp gene cause a complex and potentially fatal leukoencephalopathy are incompletely understood. Applications: Suitable for use in Western Blot and ELISA. Other applications have not been tested. Recommended Dilution: Western Blot: 1:1000 Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Shipping Conditions:: Blue Ice