Myelin protein zero (P0, MPZ) is a glycoprotein which in humans is encoded by the MPZ gene. This gene is specifically expressed in Schwann cells of the peripheral nervous system and encodes a type I transmembrane glycoprotein that is a major structural protein of the peripheral myelin sheath. The encoded protein contains a large hydrophobic extracellular domain and a smaller basic intracellular domain, which are essential for the formation and stabilization of the multilamellar structure of the compact myelin. Mutations in this gene are associated with autosomal dominant form of Charcot-Marie-Tooth disease type 1 (CMT1B) and other polyneuropathies, such as Dejerine-Sottas syndrome (DSS) and congenital hypomyelinating neuropathy (CHN). A recent study showed that two isoforms are produced from the same mRNA by use of alteative in-frame translation termination codons via a stop codon readthrough mechanism.
Formulation
0.5mg/ml if reconstituted with 0.2ml sterile DI water
Host
Rabbit
Immunogen Region
A recombinant human protein corresponding to amino acids I30-R153 was used as the immunogen for the MPZ antibody.
Isotype
IgG
Predicted Reactivity
Human
Reactivity
Human
Recombinant
No
Subcellular Location
Cytoplasmic
Uniprot
P25189
Buffer
Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide
Format
Antigen affinity purified
Purification
Antigen affinity purified
Storage
After reconstitution, the MPZ antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.