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| Cat.No | ACP03112 | Target Name | MPZ |
|---|---|---|---|
| Target Synonyms | Charcot Marie Tooth neuropathy 1B; CHM; CMT1; CMT1B; CMT2I; CMT2J; CMT4E; CMTDI3; CMTDID; DSS; HMSNIB; MPP; MPZ; Myelin peripheral protein; Myelin protein P0; Myelin protein zero; MYP0_HUMAN; P0 | Form | Liquid or Lyophilized powder |
| Expression System | E.coli | Expression Range | 30-156aa |
| Mol Weight | 18.5 kDa | Protein Length | Partial |
| Purity | Greater than 85% as determined by SDS-PAGE. | Storage Buffer | 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0. |
| Target Species | Human | Uniprot ID | P25189 |
|---|
Uniprot Id
P25189
Target Species
Human
Target Name
MPZ
Target Full Name
Myelin protein P0
Target Function
Is an adhesion molecule necessary for normal myelination in the peripheral nervous system. It mediates adhesion between adjacent myelin wraps and ultimately drives myelin compaction.
Target Involvement
Charcot-Marie-Tooth disease 1B (CMT1B); Charcot-Marie-Tooth disease 2I (CMT2I); Charcot-Marie-Tooth disease 2J (CMT2J); Adie pupil (ADIEP); Charcot-Marie-Tooth disease, dominant, intermediate type, D (CMTDID); Dejerine-Sottas syndrome (DSS); Neuropathy, congenital hypomyelinating or amyelinating (CHN); Roussy-Levy syndrome (ROULS)
Target Subcellular Location
Cell membrane; Single-pass type I membrane protein.; [Isoform L-MPZ]: Myelin membrane; Single-pass type I membrane protein.
Target Protein Families
Myelin P0 protein family
Target Tissue Specificity
Found only in peripheral nervous system Schwann cells.
Target Research Area
Neuroscience
Target Synonyms
Charcot Marie Tooth neuropathy 1B; CHM; CMT1; CMT1B; CMT2I; CMT2J; CMT4E; CMTDI3; CMTDID; DSS; HMSNIB; MPP; MPZ; Myelin peripheral protein; Myelin protein P0; Myelin protein zero; MYP0_HUMAN; P0
Target Background
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 alternative in-frame translation termination codons via a stop codon readthrough mechanism.
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