myelin protein zero

ミエリンタンパク質ゼロ

関

myelin p0 protein

WordNet

1. of or relating to the null set (a set with no members)
2. a mathematical element that when added to another number yields the same number (同)0, nought, cipher, cypher
3. adjust (as by firing under test conditions) the zero of (a gun); "He zeroed in his rifle at 200 yards" (同)zero in
4. adjust (an instrument or device) to zero value
5. the sight setting that will cause a projectile to hit the center of the target with no wind blowing
6. the point on a scale from which positive or negative numerical quantities can be measured (同)zero point
7. indicating the absence of any or all units under consideration; "a zero score" (同)0
8. having no measurable or otherwise determinable value; "the goal is zero population growth"
9. indicating an initial point or origin
10. any of a large group of nitrogenous organic compounds that are essential constituents of living cells; consist of polymers of amino acids; essential in the diet of animals for growth and for repair of tissues; can be obtained from meat and eggs and milk and legumes; "a diet high in protein"
11. a white fatty substance that forms a medullary sheath around the axis cylinder of some nerve fibers (同)myeline, medulla

PrepTutorEJDIC

1. 〈U〉(アラビア数字の)『0,零』,ゼロ / 〈U〉(温度計の)『零度』;(尺度の)零位;零点 / 〈U〉無,空(くう)(nothing) / 〈U〉最下点,どん低 / 零の,ゼロの / 〈計器など〉‘を'ゼロの目盛りに合わせる
2. 蛋白(たんばく)質

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/06/30 12:04:27」(JST)

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[Wiki en表示]

Myelin-PO_C
Identifiers
Symbol	Myelin-PO_C
Pfam	PF10570
InterPro	IPR019566
OPM superfamily	230
OPM protein	3oai
Available protein structures: Pfam structures PDB RCSB PDB; PDBe; PDBj PDBsum structure summary

Myelin protein zero
PDB rendering based on 1neu.
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 1N2P, 3OAI
Identifiers
Symbols	MPZ ; CHM; CMT1; CMT1B; CMT2I; CMT2J; CMT4E; CMTDI3; CMTDID; 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 component of plasma membrane • myelin sheath Biological process • synaptic transmission • cell death • cell-cell junction maintenance Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	4359	17528
Ensembl	ENSG00000158887	ENSMUSG00000056569
UniProt	P25189	P27573
RefSeq (mRNA)	NM_000530	NM_008623
RefSeq (protein)	NP_000521	NP_032649
Location (UCSC)	Chr 1: 161.27 – 161.28 Mb	Chr 1: 171.15 – 171.16 Mb
PubMed search	[1]	[2]

Myelin protein zero (P0, MPZ) is a glycoprotein which in humans is encoded by the MPZ gene. P0 is a major structural component of the myelin sheath, and its deficiency due to various mutations in the MPZ gene is associated with Charcot–Marie–Tooth disease and Dejerine–Sottas disease.

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, 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, 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.

Interactions

Myelin protein zero has been shown to interact with peripheral myelin protein 22.

See also

• myelin

References

Further reading

• 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. doi:10.1007/978-1-4757-9062-7_3. 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. doi:10.1212/wnl.59.5.767. PMID 12221176.  CS1 maint: Explicit use of et al. (link)
• 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.  CS1 maint: Explicit use of et al. (link)
• 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.  CS1 maint: Explicit use of et al. (link)
• 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. doi:10.1093/brain/110.1.121. PMID 3467805. 
• Tachi N, Ishikawa Y, Minami R (1985). "Two cases of congenital hypomyelination neuropathy.". Brain Dev. 6 (6): 560–5. doi:10.1016/s0387-7604(84)80101-1. 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.  CS1 maint: Explicit use of et al. (link)
• 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.  CS1 maint: Explicit use of et al. (link)
• 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.  CS1 maint: Explicit use of et al. (link)
• 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.  CS1 maint: Explicit use of et al. (link)
• 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.  CS1 maint: Explicit use of et al. (link)
• 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.  CS1 maint: Explicit use of et al. (link)
• 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. doi:10.1007/bf00206959. PMID 7527371.  CS1 maint: Explicit use of et al. (link)
• 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.  CS1 maint: Explicit use of et al. (link)
• 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.  CS1 maint: Explicit use of et al. (link)
• 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.  CS1 maint: Explicit use of et al. (link)

External links

• GeneReviews/NCBI/NIH/UW entry on Charcot-Marie-Tooth Neuropathy Type 1
• GeneReviews/NCBI/NIH/UW entry on Charcot-Marie-Tooth Neuropathy Type 2
• Myelin protein zero at the US National Library of Medicine Medical Subject Headings (MeSH)

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

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. シャルコーマリートゥース病などの一次性の遺伝性運動感覚性ニューロパチー hereditary primary motor sensory neuropathies including charcot marie tooth disease
• 2. ペリツェウス・メルツバッハー病 pelizaeus merzbacher disease
• 3. ペルオキシソーム病 peroxisomal disorders
• 4. シェーグレン・ラルソン症候群 sjogren larsson syndrome
• 5. 遺伝性ニューロパチーの概要 overview of hereditary neuropathies

English Journal

• Arf6 guanine-nucleotide exchange factor cytohesin-2 regulates myelination in nerves.

• Torii T1, Ohno N2, Miyamoto Y3, Kawahara K3, Saitoh Y4, Nakamura K3, Takashima S5, Sakagami H6, Tanoue A3, Yamauchi J7.
• Biochemical and biophysical research communications.Biochem Biophys Res Commun.2015 May 8;460(3):819-25. doi: 10.1016/j.bbrc.2015.03.113. Epub 2015 Mar 28.
• In postnatal development of the peripheral nervous system (PNS), Schwann cells differentiate to insulate neuronal axons with myelin sheaths, increasing the nerve conduction velocity. To produce the mature myelin sheath with its multiple layers, Schwann cells undergo dynamic morphological changes. Wh
• PMID 25824033

• Epigenetic patterns in successful weight loss maintainers: a pilot study.

• Huang YT1, Maccani JZ2, Hawley NL3, Wing RR4, Kelsey KT5, McCaffery JM4.
• International journal of obesity (2005).Int J Obes (Lond).2015 May;39(5):865-8. doi: 10.1038/ijo.2014.213. Epub 2014 Dec 18.
• DNA methylation changes occur in animal models of calorie restriction, simulating human dieting, and in human subjects undergoing behavioral weight loss interventions. This suggests that obese (OB) individuals may possess unique epigenetic patterns that may vary with weight loss. Here, we examine wh
• PMID 25520250

• Impaired dendritic cell function in a spontaneous autoimmune polyneuropathy.

• Quan S1, Kim HJ2, Dukala D1, Sheng JR1, Soliven B3.
• Journal of immunology (Baltimore, Md. : 1950).J Immunol.2015 May 1;194(9):4175-84. doi: 10.4049/jimmunol.1401766. Epub 2015 Mar 30.
• Spontaneous autoimmune polyneuropathy (SAP) in B7-2 knockout NOD mice mimics the progressive form of chronic inflammatory demyelinating polyradiculoneuropathy, and is mediated by myelin protein zero (P0)-reactive Th1 cells. In this study, we focused on the effect of B7-2 deletion on the function of
• PMID 25825437

Japanese Journal

• Compensatory upregulation of myelin protein zero-like 2 expression in spermatogenic cells in cell adhesion molecule-1-deficient mice

• Nakata Hiroki,Wakayama Tomohiko,Adthapanyawanich Kannika,Nishiuchi Takumi,Murakami Yoshinori,Takai Yoshimi,Iseki Shoichi
• Acta Histochemica et Cytochemica 45(1), 47-56, 2012
• … Out of the 25 genes upregulated in Cadm1-deficient mice, we took a special interest in myelin protein zero-like 2 (Mpzl2), another cell adhesion molecule of the immunoglobulin superfamily. … The levels of Mpzl2 mRNA increased by 20-fold and those of Mpzl2 protein increased by 2-fold in the testis of Cadm1-deficient mice, as analyzed with quantitative PCR and western blotting, respectively. …
• NAID 130001854317

• 髄鞘形成期における初代培養ラット後根神経節細胞への組換えアデノウイルスを用いた変異MPZ cDNAの導入

• 菊池 真,小塚 直樹,二宮 孝文 他
• 札幌医科大学保健医療学部紀要 = Bulletin of School of Health Sciences Sapporo Medical University -(13), 13-20, 2011-03-00
• NAID 40019264315

• Interleukin-6 upregulates the expression of PMP22 in cultured rat Schwann cells via a JAK2-dependent pathway

• Ito Takaaki,Ikeda Kazuo,Tomita Katsuro,Yokoyama Shigeru
• Neuroscience Letters 472(2), 104-108, 2010-03-19
• … In this study, we examined the effects of IL-6 on the expression of genes for compact myelin proteins using Schwann cell cultures prepared by multiple explantation of adult rat sciatic nerves. …
• NAID 120001939297

Related Links

• MPZ - myelin protein zero - Genetics Home Reference

The official name of this gene is “myelin protein zero.” MPZ is the gene's official symbol. The MPZ gene is also known by other names, listed below. Read more about gene names and symbols on the About page.

• MPZ myelin protein zero [Homo sapiens (human)]

This gene encodes a major structural protein of peripheral myelin. Mutations in this gene result in the autosomal dominant form of Charcot-Marie-Tooth disease type 1 and other polyneuropathies. [provided by RefSeq, Apr 2010] ... ...

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★リンクテーブル★

リンク元	「ミエリンタンパク質ゼロ」「myelin p0 protein」
関連記事	「zero」「myel」「myelin protein」

「ミエリンタンパク質ゼロ」

　　[★]

英

myelin protein zero

関

ミエリンp0タンパク質

「myelin p0 protein」

　　[★]

ミエリンp0タンパク質

関

myelin protein zero

「zero」

　　[★]

• n.

• ゼロ、零点

関

null

　 　 　 　 　 　 　 　 　 　

「myel」

　　[★]

• comb form

• 髄、脊髄、骨髄の意味

• ex.

• myeloncephalon 髄脳

「myelin protein」

　　[★]

ミエリンタンパク質