Recombinant Human Myelin Protein P0 (MPZ) Protein (His)

Beta LifeScience SKU/CAT #: BLC-03676P
Greater than 85% as determined by SDS-PAGE.
Greater than 85% as determined by SDS-PAGE.
Based on the SEQUEST from database of E.coli host and target protein, the LC-MS/MS Analysis result of this product could indicate that this peptide derived from E.coli-expressed Homo sapiens (Human) MPZ.
Based on the SEQUEST from database of E.coli host and target protein, the LC-MS/MS Analysis result of this product could indicate that this peptide derived from E.coli-expressed Homo sapiens (Human) MPZ.
Based on the SEQUEST from database of E.coli host and target protein, the LC-MS/MS Analysis result of this product could indicate that this peptide derived from E.coli-expressed Homo sapiens (Human) MPZ.
Based on the SEQUEST from database of E.coli host and target protein, the LC-MS/MS Analysis result of this product could indicate that this peptide derived from E.coli-expressed Homo sapiens (Human) MPZ.

Recombinant Human Myelin Protein P0 (MPZ) Protein (His)

Beta LifeScience SKU/CAT #: BLC-03676P
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Product Overview

Description Recombinant Human Myelin Protein P0 (MPZ) Protein (His) is produced by our E.coli expression system. This is a protein fragment.
Purity Greater than 85% as determined by SDS-PAGE.
Uniprotkb P25189
Target Symbol MPZ
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
Species Homo sapiens (Human)
Expression System E.coli
Tag N-6His
Target Protein Sequence IVVYTDREVHGAVGSRVTLHCSFWSSEWVSDDISFTWRYQPEGGRDAISIFHYAKGQPYIDEVGTFKERIQWVGDPRWKDGSIVIHNLDYSDNGTFTCDVKNPPDIVGKTSQVTLYVFEKVPTRYGV
Expression Range 30-156aa
Protein Length Partial
Mol. Weight 18.5 kDa
Research Area Neuroscience
Form Liquid or Lyophilized powder
Buffer Liquid form: default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.
Reconstitution Briefly centrifuged the vial prior to opening to bring the contents to the bottom. Reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL. It is recommended to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20°C/-80°C. The default final concentration of glycerol is 50%.
Storage 1. Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. 2. Avoid repeated freeze-thaw cycles. 3. Store working aliquots at 4°C for up to one week. 4. In general, protein in liquid form is stable for up to 6 months at -20°C/-80°C. Protein in lyophilized powder form is stable for up to 12 months at -20°C/-80°C.
Notes Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.

Target Details

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.
Subcellular Location Cell membrane; Single-pass type I membrane protein.; [Isoform L-MPZ]: Myelin membrane; Single-pass type I membrane protein.
Protein Families Myelin P0 protein family
Database References

HGNC: 7225

OMIM: 103100

KEGG: hsa:4359

STRING: 9606.ENSP00000431538

UniGene: PMID: 29465609

  • In this Chinese Han population six novel Charcot-Marie-Tooth disease-associated gene mutations of MPZ (c.440T>C) was discovered. PMID: 27862672
  • The obtained results depict that the protein with I30T mutation had variable structural conformation and dynamic behavior than native and mutant I30M of MPZ protein. PMID: 26135405
  • interaction with neurofascins impaired by mutations D6Y, D32G, and H52Y responsible for late onset forms of the human disease PMID: 26406915
  • Genotype of MPZ mutations and phenotype of Charcot Marie Tooth Disease are correlated. PMID: 26310628
  • 2 heterozygous missense mutations were identified among 38 Italian CMT2 patients. PMID: 24819634
  • P0 protein in serum may be an early effective biomarker for peripheral nerve neuropathy PMID: 24762602
  • As a result of the haplotype analysis, based on ten markers (seven SNPs, two microsatellites and an intronic polyA stretch), the founder effect hypothesis for this allele migration is suggestive. PMID: 25720167
  • This study demonistrated that The mutation c.419C>G of MPZ exhibited relatively late onset and slowly progressive CMT1 phenotype. PMID: 24028194
  • This study showed that mutation of MPZ in patient with Charcot-Marie-Tooth disease. PMID: 23743332
  • this mutation is especially important because it implicates the significance of the immunoglobulin-like structure of MPZ protein PMID: 22633464
  • MPZ-related neuropathy should be considered in the diagnostic work up of patients with painful axonal neuropathy PMID: 23279346
  • The p.Arg106Cys allele in MPZ causes late-onset predominantly axonal sensory and motor neuropathy. PMID: 22222859
  • report of 2 siblings who presented with early onset severe Charcot-Marie-Tooth disease; a novel heterozygous C to T base substitution at neucleotide position 199 (c.199C>T) was identified in exon 2 of MPZ resulting in substitution of arginine for cysteine at codon 67 (p.Arg67Cys) PMID: 23197742
  • Myelin protein zero is a key structural component of compact myelin, and over 100 mutations in this protein have been reported, which can give rise to neuropathies with either axonal, demyelinating, or other features encompassing a range of severity. PMID: 22704856
  • Patients with CMT1B caused by Ser63del MPZ have a classical CMT1 phenotype that is much less severe than that of patients with Arg98Cys MPZ PMID: 22734905
  • Thiss study demonistrated that two affected member of the same family with the same genotype had an 8-base pair deletion, c.160_167delTCCCGGGT in MPZ exon 2. PMID: 22622165
  • Myelin protein P0 is a major Aire-regulated peripheral nervous system antigen demonstrating defective tolerance to P0 in both Aire-deficient mice and humans. PMID: 22490868
  • L-MPZ, a novel isoform of myelin P0, is produced by stop codon readthrough. PMID: 22457349
  • The overall frequency of MPZ mutation was 0.58% in a Greek population of Charcot-Marie-Tooth type 1. PMID: 22243284
  • The new allelic variants of hereditary motor-sensor neuropathy caused by mutations in the MPZ (P0) gene are described. PMID: 22433810
  • This study expanded the spectrum of the MPZ mutations and revealed two disparate mechanisms of MPZ mutations associated with a typical Charcot Marie Tooth 1b phenotype PMID: 22018721
  • The crystal structure of the extracellular domain of human MPZ fused with maltose binding protein PMID: 21971831
  • MPZ plays an important role in a family with 6 affected members in 3 consecutive generations, presenting with motor and sensory demyelinating polyneuropathy. PMID: 22275255
  • Charcot-Marie-Tooth disease has been described in a large Norwegian family caused by a copy number variation in myelin protein zero. PMID: 21787890
  • The identified mutation in MPZ may be the underlying cause of Charcot-Marie-Tooth disease in this family. PMID: 21503568
  • we present here, for the first time, morphological data obtained in two sural nerve biopsies pointing to a hypomyelination-dysmyelination process in a family harboring the Pro132Leu mutation in the myelin protein zero gene PMID: 21107784
  • a rare myelin protein zero (MPZ) variant alters enhancer activity in vitro and in vivo PMID: 21179557
  • five patients with four novel MPZ mutations were identified by molecular genetic testing PMID: 20556410
  • Cells expressing mutant P(0), as compared with those expressing wild-type P(0), demonstrated variable degrees of reduction in the cell adhesiveness PMID: 20461396
  • two new MPZ mutations causing congenital hypomyelinating neuropathies; c.368_382delGCACGTTCACTTGTG (in-frame deletion of five amino acids) and c.392A>G, Asn131Ser PMID: 20621479
  • Phenotypes associated with each of the new mutations include severe hereditary motor and sensory neuropathy type III, and mild phenotype CMT1B presented with only decreased or absent reflexes, foot deformities and mild or absent lower limb atrophies PMID: 20456450
  • A novel frameshift mutation affecting the transmembrane domain (Leu144fs)in a patient with Charcot-Marie-Tooth disease presenting as with late-onset, remitting neurologic symptoms. PMID: 20516806
  • Charcot-Marie-Tooth disease with intermediate conduction velocities caused by a novel mutation in the MPZ gene. PMID: 20544920
  • The results of this study concluded that ARG98HIS MPZ mutation may cause hereditary and relatively mild and asymmetric demyelinating sensorimotor polyneuropathy PMID: 20215982
  • The index patient of this family with unusual Charcot-Marie-Tooth phenotype is found to have a missense mutation within the intracellular domain of myelin protein zero. PMID: 19882637
  • mutations in MPZ may have a role in Charcot-Marie-Tooth disease type 1B [case report] PMID: 19475438
  • Here we describe an unusual presentation of the Val102fs mutation characterized by symptoms of spinal root hypertrophy with no overt peroneal muscular atrophy. This report adds new data concerning the clinical presentations of MPZ mutations. PMID: 19906531
  • novel mutation in vocal cord paralysis PMID: 19950375
  • Findings suggest that the clinical features associated with MPZ mutations depend partly on the nature of amino acid change. PMID: 19293842
  • Data show that the CMT1Adup, GJB1, MPZ and PMP22 mutation frequencies were in the range of those described in other CMT patient collectives with different ethnical backgrounds. PMID: 19259128
  • SSCP analysis for this gene in Croatian patients PMID: 12211648
  • We suggest that axonal and demyelinating forms of CMT are not two distinct classes, but rather parts of a spectrum of genotypically related conditions, particularly with some MPZ mutations PMID: 12911457
  • DNA sequencing analysis shows the Asn131Lys mutation in the myelin protein zero gene in three members of an affected family. PMID: 12940837
  • This study demonstrates that autonomic disturbances may be one of the major clinical signs associated with CMT secondary to MPZ gene mutation in codon 124(thr124met mutation). PMID: 12948789
  • A novel mutation, Thr65Ala, in the MPZ gene in a patient with Charcot-Marie-Tooth type 1B disease with focally folded myelin. PMID: 15036333
  • Four missense mutations and one 4-base pair (bp) deletion, respectively, were identified in five patients, of which one mutation, c.173 T>A, has never been previously reported PMID: 15050444
  • MPZ gene screening should be performed for wide phenotype spectrum of Charcot-Marie-Tooth disease. PMID: 15094849
  • Chronic cough was associated with a Thr124 Met mutation. MPZ must be critical for maintenance of axonal function in addition to its role in myelin. All MPZ mutations associated with tonic pupils affect the same region of the extracellular domain. PMID: 15159512
  • A novel Thr124Lys mutation in the MPZ gene is associated with congenital neuropathy with hypomyelination. PMID: 15184631
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