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Protein Zero, Myelin (MPZ)

P0; DSS; CHM; CMT4E; CMT1; CMT1B; CMT2I; CMT2J; CMTDI3; HMSNIB; MPP; Myelin Protein Zero; Charcot-Marie-Tooth Neuropathy 1B; Myelin peripheral protein

Myelin protein-zero is the major structural protein of peripheral myelin.Hayasaka et al. (1993) stated that the MPZ gene contains 6 exons.You et al. (1991) found that the Mpz gene in the mouse, like that in the rat, contains 6 exons that span about 7 kb of genomic DNA.Myelin protein-zero is the major structural protein of peripheral myelin, accounting for more than 50% of the protein present in the sheath of peripheral nerves. Expression of the MPZ gene is restricted to Schwann cells; MPZ is not found in the CNS. An integral membrane glycoprotein of 28 kD, MPZ is thought to link adjacent lamellae and thereby stabilize the myelin assembly. The other 3 major components of myelin are myelin basic protein (MBP), myelin proteolipid protein (PLP1), and myelin-associated glycoprotein (MAG).

Organism species: Homo sapiens (Human)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPV813Hu01 Recombinant Protein Zero, Myelin (MPZ) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAV813Hu01 Monoclonal Antibody to Protein Zero, Myelin (MPZ) WB; IHC; ICC; IP.
PAV813Hu01 Polyclonal Antibody to Protein Zero, Myelin (MPZ) WB; IHC; ICC; IF.
Assay Kits CLV813Hu01 CLIA Kit for Protein Zero, Myelin (MPZ) CLIA assay for Antigen Detection
ELV813Hu01 ELISA Kit for Protein Zero, Myelin (MPZ) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Mus musculus (Mouse)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPV813Mu01 Recombinant Protein Zero, Myelin (MPZ) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAV813Mu01 Monoclonal Antibody to Protein Zero, Myelin (MPZ) WB; IHC; ICC; IP.
PAV813Mu01 Polyclonal Antibody to Protein Zero, Myelin (MPZ) WB; IHC; ICC; IF.
Assay Kits CLV813Mu01 CLIA Kit for Protein Zero, Myelin (MPZ) CLIA assay for Antigen Detection
ELV813Mu01 ELISA Kit for Protein Zero, Myelin (MPZ) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Rattus norvegicus (Rat)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPV813Ra01 Recombinant Protein Zero, Myelin (MPZ) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAV813Ra01 Monoclonal Antibody to Protein Zero, Myelin (MPZ) WB; IHC; ICC; IP.
PAV813Ra01 Polyclonal Antibody to Protein Zero, Myelin (MPZ) WB; IHC; ICC; IF.
Assay Kits CLV813Ra01 CLIA Kit for Protein Zero, Myelin (MPZ) CLIA assay for Antigen Detection
ELV813Ra01 ELISA Kit for Protein Zero, Myelin (MPZ) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Canis familiaris; Canine (Dog)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPV813Ca01 Recombinant Protein Zero, Myelin (MPZ) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAV813Ca01 Monoclonal Antibody to Protein Zero, Myelin (MPZ) WB; IHC; ICC; IP.
PAV813Ca01 Polyclonal Antibody to Protein Zero, Myelin (MPZ) WB; IHC; ICC; IF.
Assay Kits CLV813Ca01 CLIA Kit for Protein Zero, Myelin (MPZ) CLIA assay for Antigen Detection
ELV813Ca01 ELISA Kit for Protein Zero, Myelin (MPZ) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Sus scrofa; Porcine (Pig)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPV813Po01 Recombinant Protein Zero, Myelin (MPZ) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAV813Po01 Monoclonal Antibody to Protein Zero, Myelin (MPZ) WB; IHC; ICC; IP.
PAV813Po01 Polyclonal Antibody to Protein Zero, Myelin (MPZ) WB; IHC; ICC; IF.
Assay Kits CLV813Po01 CLIA Kit for Protein Zero, Myelin (MPZ) CLIA assay for Antigen Detection
ELV813Po01 ELISA Kit for Protein Zero, Myelin (MPZ) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Bos taurus; Bovine (Cattle)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPV813Bo01 Recombinant Protein Zero, Myelin (MPZ) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAV813Bo01 Monoclonal Antibody to Protein Zero, Myelin (MPZ) WB; IHC; ICC; IP.
PAV813Bo01 Polyclonal Antibody to Protein Zero, Myelin (MPZ) WB; IHC; ICC; IF.
Assay Kits CLV813Bo01 CLIA Kit for Protein Zero, Myelin (MPZ) CLIA assay for Antigen Detection
ELV813Bo01 ELISA Kit for Protein Zero, Myelin (MPZ) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Oryctolagus cuniculus (Rabbit)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPV813Rb01 Recombinant Protein Zero, Myelin (MPZ) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAV813Rb01 Monoclonal Antibody to Protein Zero, Myelin (MPZ) WB; IHC; ICC; IP.
PAV813Rb01 Polyclonal Antibody to Protein Zero, Myelin (MPZ) WB; IHC; ICC; IF.
Assay Kits CLV813Rb01 CLIA Kit for Protein Zero, Myelin (MPZ) CLIA assay for Antigen Detection
ELV813Rb01 ELISA Kit for Protein Zero, Myelin (MPZ) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Rhesus monkey (Simian)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPV813Mo01 Recombinant Protein Zero, Myelin (MPZ) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAV813Mo01 Monoclonal Antibody to Protein Zero, Myelin (MPZ) WB; IHC; ICC; IP.
PAV813Mo01 Polyclonal Antibody to Protein Zero, Myelin (MPZ) WB; IHC; ICC; IF.
Assay Kits CLV813Mo01 CLIA Kit for Protein Zero, Myelin (MPZ) CLIA assay for Antigen Detection
ELV813Mo01 ELISA Kit for Protein Zero, Myelin (MPZ) Enzyme-linked immunosorbent assay for Antigen Detection

Reference

"Isolation and sequence determination of cDNA encoding the major structural protein of human peripheral myelin."
Biochem. Biophys. Res. Commun. 180:515-518(1991) [PubMed] [Europe PMC] [Abstract]

"Mutation of the myelin P0 gene in Charcot-Marie-Tooth neuropathy type 1."
Biochem. Biophys. Res. Commun. 194:1317-1322(1993) [PubMed] [Europe PMC] [Abstract]

"The major peripheral myelin protein zero gene: structure and localization in the cluster of Fc gamma receptor genes on human chromosome 1q21.3-q23."
Hum. Mol. Genet. 2:2051-2054(1993) [PubMed] [Europe PMC] [Abstract]

"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]

"The DNA sequence and biological annotation of human chromosome 1."
Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract]

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"Deletion of the serine 34 codon from the major peripheral myelin protein P0 gene in Charcot-Marie-Tooth disease type 1B."
Nat. Genet. 5:35-39(1993) [PubMed] [Europe PMC] [Abstract]

"Linkage and mutation analysis in an extended family with Charcot-Marie-Tooth disease type 1B."
J. Med. Genet. 31:811-815(1994) [PubMed] [Europe PMC] [Abstract]

"L-MPZ, a novel isoform of myelin P0, is produced by stop codon readthrough."
J. Biol. Chem. 287:17765-17776(2012) [PubMed] [Europe PMC] [Abstract]

"Molecular genetics of Charcot-Marie-Tooth neuropathy."
Adv. Hum. Genet. 22:117-152(1994) [PubMed] [Europe PMC] [Abstract]

"Charcot-Marie-Tooth disease: a new paradigm for the mechanism of inherited disease."
Trends Genet. 10:128-133(1994) [PubMed] [Europe PMC] [Abstract]

"Mutations in the peripheral myelin genes and associated genes in inherited peripheral neuropathies."
Hum. Mutat. 13:11-28(1999) [PubMed] [Europe PMC] [Abstract]

"Crystal structure of the extracellular domain of human myelin protein zero."
Proteins 80:307-313(2012) [PubMed] [Europe PMC] [Abstract]

"Mutation of the myelin P0 gene in Charcot-Marie-Tooth neuropathy type 1B."
Hum. Mol. Genet. 2:1369-1372(1993) [PubMed] [Europe PMC] [Abstract]

"New mutation of the myelin P0 gene in a pedigree of Charcot-Marie-Tooth neuropathy 1."
Biochem. Mol. Biol. Int. 31:169-173(1993) [PubMed] [Europe PMC] [Abstract]

"Charcot-Marie-Tooth neuropathy type 1B is associated with mutations of the myelin P0 gene."
Nat. Genet. 5:31-34(1993) [PubMed] [Europe PMC] [Abstract]

"Myelin protein zero gene mutated in Charcot-Marie-Tooth type 1B patients."
Proc. Natl. Acad. Sci. U.S.A. 90:10856-10860(1993) [PubMed] [Europe PMC] [Abstract]

"De novo mutation of the myelin P0 gene in Dejerine-Sottas disease (hereditary motor and sensory neuropathy type III)."
Nat. Genet. 5:266-268(1993) [PubMed] [Europe PMC] [Abstract]

"Rapid screening of myelin genes in CMT1 patients by SSCP analysis: identification of new mutations and polymorphisms in the P0 gene."
Hum. Genet. 94:653-657(1994) [PubMed] [Europe PMC] [Abstract]

"Charcot-Marie-Tooth type 1B neuropathy: third mutation of serine 63 codon in the major peripheral myelin glycoprotein PO gene."
Clin. Genet. 48:281-283(1995) [PubMed] [Europe PMC] [Abstract]

"Mutations in the myelin protein zero gene associated with Charcot-Marie-Tooth disease type 1B."
Hum. Mutat. 6:50-54(1995) [PubMed] [Europe PMC] [Abstract]

"A novel homozygous mutation of the myelin Po gene producing Dejerine-Sottas disease (hereditary motor and sensory neuropathy type III)."
Biochem. Biophys. Res. Commun. 222:107-110(1996) [PubMed] [Europe PMC] [Abstract]

"Myelin protein zero (MPZ) gene mutations in nonduplication type 1 Charcot-Marie-Tooth disease."
Hum. Mutat. 7:36-45(1996) [PubMed] [Europe PMC] [Abstract]

"Charcot-Marie-Tooth type 1B neuropathy: a mutation at the single glycosylation site in the major peripheral myelin glycoprotein Po."
Hum. Mutat. 8:185-186(1996) [PubMed] [Europe PMC] [Abstract]

"Two divergent types of nerve pathology in patients with different P0 mutations in Charcot-Marie-Tooth disease."
Neurology 47:761-765(1996) [PubMed] [Europe PMC] [Abstract]

"Clinical phenotypes of different MPZ (P0) mutations may include Charcot-Marie-Tooth type 1B, Dejerine-Sottas, and congenital hypomyelination."
Neuron 17:451-460(1996) [PubMed] [Europe PMC] [Abstract]

"Novel mutation of the myelin Po gene in a pedigree with Charcot-Marie-Tooth disease type 1B."
Am. J. Med. Genet. 71:246-248(1997) [PubMed] [Europe PMC] [Abstract]

"Mutational analysis of the MPZ, PMP22 and Cx32 genes in patients of Spanish ancestry with Charcot-Marie-Tooth disease and hereditary neuropathy with liability to pressure palsies."
Hum. Genet. 99:746-754(1997) [PubMed] [Europe PMC] [Abstract]

"Novel mutation of the myelin P0 gene in a CMT1B family."
Hum. Mutat. 9:74-77(1997) [PubMed] [Europe PMC] [Abstract]

"Multiple de novo MPZ (P0) point mutations in a sporadic Dejerine-Sottas case."
Hum. Mutat. 10:21-24(1997) [PubMed] [Europe PMC] [Abstract]

"De novo mutation of the myelin P0 gene in Dejerine-Sottas disease (hereditary motor and sensory neuropathy type III): two amino acid insertion after Asp 118."
Hum. Mutat. Suppl. 1:S103-S105(1998) [PubMed] [Europe PMC] [Abstract]

"Mutations of the same sequence of the myelin P0 gene causing two different phenotypes."
Hum. Mutat. Suppl. 1:S217-S219(1998) [PubMed] [Europe PMC] [Abstract]

"Mutation analysis in Charcot-Marie-Tooth disease type 1 (CMT1)."
Hum. Mutat. Suppl. 1:S242-S247(1998) [PubMed] [Europe PMC] [Abstract]

"Spectrum of mutations in Finnish patients with Charcot-Marie-Tooth disease and related neuropathies."
Hum. Mutat. 12:59-68(1998) [PubMed] [Europe PMC] [Abstract]

"Charcot-Marie-Tooth disease type 2 associated with mutation of the myelin protein zero gene."
Neurology 50:1397-1401(1998) [PubMed] [Europe PMC] [Abstract]

"The Roussy-Levy family: from the original description to the gene."
Ann. Neurol. 46:770-773(1999) [PubMed] [Europe PMC] [Abstract]

"The Thr124Met mutation in the peripheral myelin protein zero (MPZ) gene is associated with a clinically distinct Charcot-Marie-Tooth phenotype."
Brain 122:281-290(1999) [PubMed] [Europe PMC] [Abstract]

"Novel mutations of the myelin P0 gene in two Charcot-Marie-Tooth type 1 patients from Turkey."
Eur. J. Hum. Genet. Suppl. 7:116-116(1999)

"Axonal phenotype of Charcot-Marie-Tooth disease associated with a mutation in the myelin protein zero gene."
J. Neurol. Neurosurg. Psych. 66:779-782(1999) [PubMed] [Europe PMC] [Abstract]

"Novel mutation in the myelin protein zero gene in a family with intermediate hereditary motor and sensory neuropathy."
J. Neurol. Neurosurg. Psych. 67:174-179(1999) [PubMed] [Europe PMC] [Abstract]

"A novel MPZ gene mutation in dominantly inherited neuropathy with focally folded myelin sheaths."
Neurology 52:1271-1275(1999) [PubMed] [Europe PMC] [Abstract]

"Peripheral myelin modification in CMT1B correlates with MPZ gene mutations."
Neuromuscul. Disord. 9:361-367(1999) [PubMed] [Europe PMC] [Abstract]

"Focally folded myelin in Charcot-Marie-Tooth neuropathy type 1B with Ser49Leu in the myelin protein zero."
Acta Neuropathol. 100:299-304(2000) [PubMed] [Europe PMC] [Abstract]

"Charcot-Marie-Tooth neuropathy type 2 and P0 point mutations: two novel amino acid substitutions (Asp61Gly; Tyr119Cys) and a possible 'hotspot' on Thr124Met."
Brain Pathol. 10:235-248(2000) [PubMed] [Europe PMC] [Abstract]

"Screening for mutations in the peripheral myelin genes PMP22, MPZ and Cx32 (GJB1) in Russian Charcot-Marie-Tooth neuropathy patients."
Hum. Mutat. 15:340-347(2000) [PubMed] [Europe PMC] [Abstract]

"Mutations in the peripheral myelin protein zero and connexin32 genes detected by non-isotopic RNase cleavage assay and their phenotypes in Japanese patients with Charcot-Marie-Tooth disease."
Hum. Mutat. 16:177-178(2000) [PubMed] [Europe PMC] [Abstract]

"An axonal form of Charcot-Marie-Tooth disease showing distinctive features in association with mutations in the peripheral myelin protein zero gene (Thr124Met or Asp75Val)."
J. Neurol. Neurosurg. Psych. 69:806-811(2000) [PubMed] [Europe PMC] [Abstract]

"Charcot-Marie-Tooth disease type I and related demyelinating neuropathies: mutation analysis in a large cohort of Italian families."
Hum. Mutat. 18:32-41(2001) [PubMed] [Europe PMC] [Abstract]

"Mutation analysis in Chariot-Marie Tooth disease type 1: point mutations in the MPZ gene and the GJB1 gene cause comparable phenotypic heterogeneity."
J. Neurol. 248:410-415(2001) [PubMed] [Europe PMC] [Abstract]

"The range of chronic demyelinating neuropathy of infancy: a clinico-pathological and genetic study of 15 unrelated cases."
J. Neurol. 248:795-803(2001) [PubMed] [Europe PMC] [Abstract]

"A somatic and germline mosaic mutation in MPZ/P(0) mimics recessive inheritance of CMT1B."
Neurology 57:101-105(2001) [PubMed] [Europe PMC] [Abstract]

"Charcot-Marie-Tooth disease and related neuropathies: mutation distribution and genotype-phenotype correlation."
Ann. Neurol. 51:190-201(2002) [PubMed] [Europe PMC] [Abstract]

"Molecular analysis in Japanese patients with Charcot-Marie-Tooth disease: DGGE analysis for PMP22, MPZ, and Cx32/GJB1 mutations."
Hum. Mutat. 20:392-398(2002) [PubMed] [Europe PMC] [Abstract]

"Corticosteroid-responsive asymmetric neuropathy with a myelin protein zero gene mutation."
Neurology 59:767-769(2002) [PubMed] [Europe PMC] [Abstract]

"Two amino-acid substitutions in the myelin protein zero gene of a case of Charcot-Marie-Tooth disease associated with light-near dissociation."
Neuromuscul. Disord. 12:281-285(2002) [PubMed] [Europe PMC] [Abstract]

"Charcot-Marie-Tooth neuropathy: clinical phenotypes of four novel mutations in the MPZ and Cx 32 genes."
Neuromuscul. Disord. 12:643-650(2002) [PubMed] [Europe PMC] [Abstract]

"Demyelinating and axonal features of Charcot-Marie-Tooth disease with mutations of myelin-related proteins (PMP22, MPZ and Cx32): a clinicopathological study of 205 Japanese patients."
Brain 126:134-151(2003) [PubMed] [Europe PMC] [Abstract]

"Novel mutations in the Charcot-Marie-Tooth disease genes PMP22, MPZ, and GJB1."
Hum. Mutat. 21:100-100(2003) [PubMed] [Europe PMC] [Abstract]

"Clinical and genetic analysis of CMT1B in a Nigerian family."
Muscle Nerve 27:628-630(2003) [PubMed] [Europe PMC] [Abstract]

"Charcot-Marie-Tooth disease: a novel Tyr145Ser mutation in the myelin protein zero (MPZ, P0) gene causes different phenotypes in homozygous and heterozygous carriers within one family."
Neurogenetics 4:191-197(2003) [PubMed] [Europe PMC] [Abstract]

"Late onset Charcot-Marie-Tooth 2 syndrome caused by two novel mutations in the MPZ gene."
Neurology 61:1435-1437(2003) [PubMed] [Europe PMC] [Abstract]

"Phenotypic clustering in MPZ mutations."
Brain 127:371-384(2004) [PubMed] [Europe PMC] [Abstract]

"An axonal form of Charcot-Marie-Tooth disease with a novel missense mutation in the myelin protein zero gene."
J. Peripher. Nerv. Syst. 9:1-2(2004) [PubMed] [Europe PMC] [Abstract]

"Mutational analysis of PMP22, MPZ, GJB1, EGR2 and NEFL in Korean Charcot-Marie-Tooth neuropathy patients."
Hum. Mutat. 24:185-186(2004) [PubMed] [Europe PMC] [Abstract]

"A novel MPZ gene mutation in congenital neuropathy with hypomyelination."
Neurology 62:2122-2123(2004) [PubMed] [Europe PMC] [Abstract]

"Hearing loss as the first feature of late-onset axonal CMT disease due to a novel P0 mutation."
Neurology 63:733-735(2004) [PubMed] [Europe PMC] [Abstract]

"Gene dosage sensitivity of a novel mutation in the intracellular domain of P0 associated with Charcot-Marie-Tooth disease type 1B."
Neuromuscul. Disord. 16:183-187(2006) [PubMed] [Europe PMC] [Abstract]

"Different cellular and molecular mechanisms for early and late-onset myelin protein zero mutations."
Hum. Mol. Genet. 17:1877-1889(2008) [PubMed] [Europe PMC] [Abstract]

"Chronic cough due to Thr124Met mutation in the peripheral myelin protein zero (MPZ gene)."
Neurology 62:1905-1906(2004) [PubMed] [Europe PMC] [Abstract]

"A novel mutation, Thr65Ala, in the MPZ gene in a patient with Charcot-Marie-Tooth type 1B disease with focally folded myelin."
Neuromuscul. Disord. 14:229-232(2004) [PubMed] [Europe PMC] [Abstract]

"Case records of the Massachusetts General Hospital. Case 18-2006. A 57-year-old woman with numbness and weakness of the feet and legs."
N. Engl. J. Med. 354:2584-2592(2006) [PubMed] [Europe PMC] [Abstract]