Myelin protein zero

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Myelin protein zero

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Myelin-PO_C

Identifiers

Symbol

Myelin-PO_C

Available protein structures:
Pfam	structures
PDB	RCSB PDB; PDBe
PDBsum	structure summary

Myelin protein zero

PDB rendering based on 1neu.

Available structures

PDB

Ortholog search: PDBe, RCSB

List of PDB id codes
3OAI

Identifiers

Symbols

MPZ; CHM; CMT1; CMT1B; CMT2I; CMT2J; CMT4E; CMTDI3; DSS; HMSNIB; MPP; P0

External IDs

OMIM: 159440 MGI: 103177 HomoloGene: 445 GeneCards: MPZ Gene

Gene Ontology
Molecular function	• structural molecule activity
Cellular component	• plasma membrane • integral to plasma membrane
Biological process	• synaptic transmission
Sources: Amigo / QuickGO

RNA expression pattern

More reference expression data

Orthologs

Species

Human

Mouse

RefSeq (mRNA)

RefSeq (protein)

Location (UCSC)

Chr 1:
161.27 – 161.28 Mb

Chr 1:
173.08 – 173.09 Mb

PubMed search

[1]

[2]

Myelin protein zero (P0) is a glycoprotein associated with Charcot-Marie-Tooth disease and Dejerine-Sottas disease.

Myelin protein-zero is the major structural protein of peripheral myelin.[supplied by OMIM]^[1]

Contents

1 Function
2 Interactions
3 See also
4 References
5 Further reading
6 External links

Function

The myelin sheath is a multi-layered membrane, unique to the nervous system, that functions as an insulator to greatly increase the velocity of axonal impulse conduction. The P0 glycoprotein, absent in the central nervous system,^[2] is a major component of the myelin sheath in peripheral nerves. It comprises a large extracellular N-terminal domain, a single transmembrane (TM) region, and a smaller positively charged intracellular domain. It is postulated that P0 is a structural element in the formation and stabilisation of peripheral nerve myelin,^[3] holding its characteristic coil structure together by the interaction of its positively-charged domain with acidic lipids in the cytoplasmic face of the opposed bilayer, and by interaction between hydrophobic globular `heads' of adjacent extracellular domains.^[2]

Interactions

Myelin protein zero has been shown to interact with Peripheral myelin protein 22.^[4]

References

^ "Entrez Gene: MPZ myelin protein zero (Charcot-Marie-Tooth neuropathy 1B)". http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=4359.
^ ^a ^b Sakamoto Y, Kitamura K, Yoshimura K, Nishijima T, Uyemura K (March 1987). "Complete amino acid sequence of PO protein in bovine peripheral nerve myelin". J. Biol. Chem. 262 (9): 4208-14. PMID 2435734.
^ Lemke G, Axel R (March 1985). "Isolation and sequence of a cDNA encoding the major structural protein of peripheral myelin". Cell 40 (3): 501-8. PMID 2578885.
^ D'Urso, D; Ehrhardt P, Müller H W (May. 1999). "Peripheral myelin protein 22 and protein zero: a novel association in peripheral nervous system myelin". J. Neurosci. (UNITED STATES) 19 (9): 3396–403. ISSN 0270-6474. PMID 10212299.

Patel PI, Lupski JR (1994). "Charcot-Marie-Tooth disease: a new paradigm for the mechanism of inherited disease.". Trends Genet. 10 (4): 128–33. doi:10.1016/0168-9525(94)90214-3. PMID 7518101.
Roa BB, Lupski JR (1995). "Molecular genetics of Charcot-Marie-Tooth neuropathy.". Adv. Hum. Genet. 22: 117–52. PMID 7762451.
Nelis E, Haites N, Van Broeckhoven C (1999). "Mutations in the peripheral myelin genes and associated genes in inherited peripheral neuropathies.". Hum. Mutat. 13 (1): 11–28. doi:10.1002/(SICI)1098-1004(1999)13:1<11::AID-HUMU2>3.0.CO;2-A. PMID 9888385.
Watanabe M, Yamamoto N, Ohkoshi N, et al. (2002). "Corticosteroid- responsive asymmetric neuropathy with a myelin protein zero gene mutation.". Neurology 59 (5): 767–9. PMID 12221176.
Hattori N, Yamamoto M, Yoshihara T, et al. (2003). "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 (Pt 1): 134–51. doi:10.1093/brain/awg012. PMID 12477701.
Shy ME (2006). "Peripheral neuropathies caused by mutations in the myelin protein zero.". J. Neurol. Sci. 242 (1-2): 55–66. doi:10.1016/j.jns.2005.11.015. PMID 16414078.
Hayasaka K, Nanao K, Tahara M, et al. (1991). "Isolation and sequence determination of cDNA encoding the major structural protein of human peripheral myelin.". Biochem. Biophys. Res. Commun. 180 (2): 515–8. doi:10.1016/S0006-291X(05)81094-0. PMID 1719967.
Ouvrier RA, McLeod JG, Conchin TE (1987). "The hypertrophic forms of hereditary motor and sensory neuropathy. A study of hypertrophic Charcot-Marie-Tooth disease (HMSN type I) and Dejerine-Sottas disease (HMSN type III) in childhood.". Brain 110 (1): 121–48. PMID 3467805.
Tachi N, Ishikawa Y, Minami R (1985). "Two cases of congenital hypomyelination neuropathy.". Brain Dev. 6 (6): 560–5. PMID 6099985.
Hayasaka K, Himoro M, Wang Y, et al. (1993). "Structure and chromosomal localization of the gene encoding the human myelin protein zero (MPZ).". Genomics 17 (3): 755–8. doi:10.1006/geno.1993.1400. PMID 7503936.
Su Y, Brooks DG, Li L, et al. (1993). "Myelin protein zero gene mutated in Charcot-Marie-tooth type 1B patients.". Proc. Natl. Acad. Sci. U.S.A. 90 (22): 10856–60. doi:10.1073/pnas.90.22.10856. PMC 47877. PMID 7504284. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=47877.
Himoro M, Yoshikawa H, Matsui T, et al. (1994). "New mutation of the myelin P0 gene in a pedigree of Charcot-Marie-Tooth neuropathy 1.". Biochem. Mol. Biol. Int. 31 (1): 169–73. PMID 7505151.
Hayasaka K, Himoro M, Sawaishi Y, et al. (1994). "De novo mutation of the myelin P0 gene in Dejerine-Sottas disease (hereditary motor and sensory neuropathy type III).". Nat. Genet. 5 (3): 266–8. doi:10.1038/ng1193-266. PMID 7506095.
Pham-Dinh D, Fourbil Y, Blanquet F, et al. (1994). "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 (12): 2051–4. doi:10.1093/hmg/2.12.2051. PMID 7509228.
Thomas FP, Lebo RV, Rosoklija G, et al. (1994). "Tomaculous neuropathy in chromosome 1 Charcot-Marie-Tooth syndrome.". Acta Neuropathol. 87 (1): 91–7. doi:10.1007/BF00386259. PMID 7511317.
Nelis E, Timmerman V, De Jonghe P, et al. (1995). "Rapid screening of myelin genes in CMT1 patients by SSCP analysis: identification of new mutations and polymorphisms in the P0 gene.". Hum. Genet. 94 (6): 653–7. PMID 7527371.
Hilmi S, Fournier M, Valeins H, et al. (1995). "Myelin P0 glycoprotein: identification of the site phosphorylated in vitro and in vivo by endogenous protein kinases.". J. Neurochem. 64 (2): 902–7. doi:10.1046/j.1471-4159.1995.64020902.x. PMID 7530295.
Rautenstrauss B, Nelis E, Grehl H, et al. (1995). "Identification of a de novo insertional mutation in P0 in a patient with a Déjérine-Sottas syndrome (DSS) phenotype.". Hum. Mol. Genet. 3 (9): 1701–2. doi:10.1093/hmg/3.9.1701. PMID 7530550.
Latour P, Blanquet F, Nelis E, et al. (1995). "Mutations in the myelin protein zero gene associated with Charcot-Marie-Tooth disease type 1B.". Hum. Mutat. 6 (1): 50–4. doi:10.1002/humu.1380060110. PMID 7550231.

PDB gallery

1neu: STRUCTURE OF MYELIN MEMBRANE ADHESION MOLECULE P0

External links

Look up myelin protein zero in Wiktionary, the free dictionary.

Protein, glycoconjugate: glycoproteins and glycopeptides

Mucoproteins

Mucin	CD43 · CD164 · MUC1 · MUC2 · MUC3A · MUC3B · MUC4 · MUC5AC · MUC5B · MUC6 · MUC7 · MUC8 · MUC12 · MUC13 · MUC15 · MUC16 · MUC17 · MUC19 · MUC20

Other	Haptoglobin · Intrinsic factor · Orosomucoid · Peptidoglycan · Phytohaemagglutinin · Ovomucin

Proteoglycans

CS/DS	Decorin · Biglycan · Versican

HS/CS	Testican · Perlecan

CS	Chondroitin sulfate proteoglycans: Aggrecan · Neurocan · Brevican · CD44 · CSPG4 · CSPG5 · Platelet factor 4 · Structural maintenance of chromosomes 3

KS	Fibromodulin · Lumican · Keratocan

HS	Syndecan 1

Other

Activin and inhibin · ADAM · Alpha 1-antichymotrypsin · Apolipoprotein H · CD70 · Asialoglycoprotein · Avidin · B-cell activating factor · 4-1BB ligand · Cholesterylester transfer protein · Clusterin · Colony-stimulating factor · Hemopexin · Lactoferrin · Membrane glycoproteins · Myelin protein zero · Osteonectin · Protein C · Protein S · Serum amyloid P component · Sialoglycoprotein (CD43, Glycophorin, Glycophorin C) · Thrombopoietin · Thyroglobulin · Thyroxine-binding proteins · Transcortin · Tumor necrosis factor-alpha · Uteroglobin · Vitronectin

M: MET

mt, k, c/g/r/p/y/i, f/h/s/l/o/e, a/u, n, m

k, cgrp/y/i, f/h/s/l/o/e, au, n, m, epon

m(A16/C10),i(k, c/g/r/p/y/i, f/h/s/o/e, a/u, n, m)

biochemical families: proteins (amino acids/intermediates) · nucleic acids (constituents/intermediates) · carbohydrates (glycoproteins, alcohols, glycosides)
lipids (fatty acids/intermediates, phospholipids, steroids, sphingolipids, eicosanoids) · tetrapyrroles/intermediates

Membrane proteins: cell adhesion molecules

Calcium-independent

IgSF CAM	N-CAM (Myelin protein zero) · ICAM (1, 5) · VCAM-1 · PE-CAM · L1-CAM · Nectin (PVRL1, PVRL2, PVRL3)

Integrins	LFA-1 (CD11a+CD18) · Integrin alphaXbeta2 (CD11c+CD18) · Macrophage-1 antigen (CD11b+CD18) · VLA-4 (CD49d+CD29) · Glycoprotein IIb/IIIa (ITGA2B+ITGB3)

Calcium-dependent

Cadherins

Classical	CDH1 · CDH2 · CDH3

Desmosomal	Desmoglein (DSG1, DSG2, DSG3, DSG4) · Desmocollin (DSC1, DSC2, DSC3)

Protocadherin	PCDH1

Unconventional/ungrouped	T-cadherin · CDH4 · CDH5 · CDH6 · CDH8 · CDH11 · CDH12 · CDH15 · CDH16 · CDH17 · CDH9 · CDH10

Selectins

E-selectin · L-selectin · P-selectin

Other

Lymphocyte homing receptor: CD44 · L-selectin · integrin (VLA-4, LFA-1)

Carcinoembryonic antigen · CD22 · CD24 · CD44 · CD146 · CD164

see also cell membrane protein disorders
B memb: cead, trns (1A, 1C, 1F, 2A, 3A1, 3A2-3, 3D), othr

v t e Protein: cell membrane proteins (other than Cell surface receptor, enzymes, and cytoskeleton)

Arrestin	SAG, ARRB1, ARRB2, ARR3

Membrane-spanning 4A	MS4A1 · MS4A2 · MS4A3 · MS4A4A · MS4A4E · MS4A5 · MS4A6A · MS4A6E · MS4A7 · MS4A8B · MS4A9 · MS4A10 · MS4A12 · MS4A13 · MS4A14 · MS4A15 · MS4A18

Myelin	Myelin basic protein (PMP2) · Myelin proteolipid protein (PLP1) · Myelin oligodendrocyte glycoprotein · Myelin-associated glycoprotein · Myelin protein zero

Pulmonary surfactant	Pulmonary surfactant-associated protein B · Pulmonary surfactant-associated protein C

Tetraspanin	TSPAN1 · TSPAN2 · TSPAN3 · TSPAN4 · TSPAN5 · TSPAN6 · TSPAN7 · TSPAN8 · TSPAN9 · TSPAN10 · TSPAN11 · TSPAN12 · TSPAN13 · TSPAN14 · TSPAN15 · TSPAN16 · TSPAN17 · TSPAN18 · TSPAN19 · TSPAN20 · TSPAN21 · TSPAN22 · TSPAN23 · TSPAN24 · TSPAN25 · TSPAN26 · TSPAN27 · TSPAN28 · TSPAN29 · TSPAN30 · TSPAN31 · TSPAN32 · TSPAN33 · TSPAN34

Other/ungrouped	Calnexin · LDL-receptor-related protein associated protein · Neurofibromin 2 · Presenilin (PSEN1, PSEN2) · HFE · Phospholipid transfer proteins · Dysferlin · STRC · OTOF

see also other cell membrane protein disorders B memb: cead, trns (1A, 1C, 1F, 2A, 3A1, 3A2-3, 3D), othr

This article incorporates text from the public domain Pfam and InterPro IPR019566

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