catalog number :
MBS954588
products type :
Recombinant Protein
products full name :
Recombinant Human Myelin proteolipid protein (PLP1)
products short name :
Myelin proteolipid protein (PLP1)
products name syn :
Recombinant Myelin proteolipid protein (PLP1); Myelin proteolipid protein; PLP; Lipophilin
other names :
myelin proteolipid protein isoform 1; Myelin proteolipid protein; myelin proteolipid protein; lipophilin; major myelin proteolipid protein; proteolipid protein 1; Lipophilin
products gene name syn :
PLP1; PLP
other gene names :
PLP1; PLP1; PLP; PMD; HLD1; MMPL; SPG2; PLP/DM20; PLP; PLP
uniprot entry name :
MYPR_HUMAN
host :
E Coli or Yeast or Baculovirus or Mammalian Cell
sequence positions :
143-197, Partial.
sequence :
GKWLGHPDKFVGITYALTVVWLLVFACSAVPVYIYFNTW
TTCQSIAFPSKTSASI
form :
Liquid containing glycerol; lyophilization may be available upon request.
storage stability :
Store at -20 degree C. For extended storage, store at -20 or -80 degree C. Notes: Repeated freezing and thawing is not recommended. Store working aliquots at 4 degree C for up to one week.
other info1 :
Species: Homo sapiens (Human)
products description :
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.
ncbi acc num :
NP_000524.3
ncbi gb acc num :
NM_000533.3
ncbi summary :
This gene encodes a transmembrane proteolipid protein that is the predominant myelin protein present in the central nervous system. It may play a role in the compaction, stabilization, and maintenance of myelin sheaths, as well as in oligodendrocyte development and axonal survival. Mutations in this gene cause X-linked Pelizaeus-Merzbacher disease and spastic paraplegia type 2. Alternatively spliced transcript variants encoding distinct isoforms or having different 5' UTRs, have been identified for this gene. [provided by RefSeq, Jul 2008]
uniprot summary :
PLP1: 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. Defects in PLP1 are the cause of leukodystrophy hypomyelinating type 1 (HLD1); also known as Pelizaeus-Merzbacher disease. HLD1 is an X-linked recessive dysmyelinating disorder of the central nervous system in which myelin is not formed properly. It is characterized clinically by nystagmus, spastic quadriplegia, ataxia, and developmental delay. Defects in PLP1 are the cause of spastic paraplegia X- linked type 2 (SPG2). SPG2 is characterized by spastic gait and hyperreflexia. In some patients, complicating features include nystagmus, dysarthria, sensory disturbance, mental retardation, optic atrophy. Belongs to the myelin proteolipid protein family. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Membrane protein, multi-pass; Membrane protein, integral; Cell surface. Chromosomal Location of Human Ortholog: Xq22. Cellular Component: plasma membrane; integral to membrane; myelin sheath. Molecular Function: structural constituent of myelin sheath; structural molecule activity. Biological Process: integrin-mediated signaling pathway; synaptic transmission; substantia nigra development; myelination in the central nervous system; cell maturation; long-chain fatty acid biosynthetic process; axon ensheathment; inflammatory response; astrocyte development. Disease: Spastic Paraplegia 2, X-linked; Pelizaeus-merzbacher Disease
size2 :
0.05 mg (Baculovirus)
size3 :
0.05 mg (Mammalian-Cell)