myelin proteolipid protein

ミエリンプロテオリピドタンパク質

WordNet

1. 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"
2. a white fatty substance that forms a medullary sheath around the axis cylinder of some nerve fibers (同)myeline, medulla

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1. 蛋白(たんばく)質

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/07/21 00:41:11」(JST)

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Myelin proteolipid protein (PLP or lipophilin)
Identifiers
Symbol	Myelin_PLP
Pfam	PF01275
InterPro	IPR001614
SMART	SM00002
PROSITE	PDOC00497
Available protein structures: Pfam structures PDB RCSB PDB; PDBe; PDBj PDBsum structure summary

Myelin proteolipid protein (PLP or lipophilin) is the major myelin protein from the central nervous system (CNS). It plays an important role in the formation or maintenance of the multilamellar structure of myelin. 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.

In man point mutations in PLP are the cause of Pelizaeus-Merzbacher disease (PMD), a neurologic disorder of myelin metabolism. In animals dismyelinating diseases such as mouse 'jimpy' or dog 'shaking pup' are also caused by mutations in PLP.

PLP is a highly conserved hydrophobic protein of 276 to 280 amino acids which seems to contain four transmembrane segments, two disulfide bonds and which covalently binds lipids (at least six palmitate groups in mammals). PLP is highly related to GPM6A, a neuronal membrane glycoprotein.

Human proteins containing this domain

GPM6A; GPM6B; PLP1;

References

This article incorporates text from the public domain Pfam and InterPro IPR001614

UpToDate Contents

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• 1. ペリツェウス・メルツバッハー病 pelizaeus merzbacher disease
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• 5. フェニルケトン尿症の概要 overview of phenylketonuria

English Journal

• Assessing white matter ischemic damage in dementia patients by measurement of myelin proteins.

• Barker R, Wellington D, Esiri MM, Love S.SourceDementia Research Group, Institute of Clinical Neurosciences, School of Clinical Sciences, University of Bristol, Bristol, UK.
• Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.J Cereb Blood Flow Metab.2013 Jul;33(7):1050-7. doi: 10.1038/jcbfm.2013.46. Epub 2013 Mar 27.
• White matter ischemia is difficult to quantify histologically. Myelin-associated glycoprotein (MAG) is highly susceptible to ischemia, being expressed only adaxonally, far from the oligodendrocyte cell body. Myelin-basic protein (MBP) and proteolipid protein (PLP) are expressed throughout the myelin
• PMID 23532085

• Lamin B1 mediates cell-autonomous neuropathology in a leukodystrophy mouse model.

• Heng MY, Lin ST, Verret L, Huang Y, Kamiya S, Padiath QS, Tong Y, Palop JJ, Huang EJ, Ptácχek LJ, Fu YH.AbstractAdult-onset autosomal-dominant leukodystrophy (ADLD) is a progressive and fatal neurological disorder characterized by early autonomic dysfunction, cognitive impairment, pyramidal tract and cerebellar dysfunction, and white matter loss in the central nervous system. ADLD is caused by duplication of the LMNB1 gene, which results in increased lamin B1 transcripts and protein expression. How duplication of LMNB1 leads to myelin defects is unknown. To address this question, we developed a mouse model of ADLD that overexpresses lamin B1. These mice exhibited cognitive impairment and epilepsy, followed by age-dependent motor deficits. Selective overexpression of lamin B1 in oligodendrocytes also resulted in marked motor deficits and myelin defects, suggesting these deficits are cell autonomous. Proteomic and genome-wide transcriptome studies indicated that lamin B1 overexpression is associated with downregulation of proteolipid protein, a highly abundant myelin sheath component that was previously linked to another myelin-related disorder, Pelizaeus-Merzbacher disease. Furthermore, we found that lamin B1 overexpression leads to reduced occupancy of Yin Yang 1 transcription factor at the promoter region of proteolipid protein. These studies identify a mechanism by which lamin B1 overexpression mediates oligodendrocyte cell-autonomous neuropathology in ADLD and implicate lamin B1 as an important regulator of myelin formation and maintenance during aging.
• The Journal of clinical investigation.J Clin Invest.2013 Jun 3;123(6):2719-29. doi: 10.1172/JCI66737. Epub 2013 May 15.
• Adult-onset autosomal-dominant leukodystrophy (ADLD) is a progressive and fatal neurological disorder characterized by early autonomic dysfunction, cognitive impairment, pyramidal tract and cerebellar dysfunction, and white matter loss in the central nervous system. ADLD is caused by duplication of
• PMID 23676464

• Active Suppression Induced by Repetitive Self-Epitopes Protects against EAE Development.

• Puentes F, Dickhaut K, Hofstätter M, Falk K, Rötzschke O.SourceMax-Delbrück-Center for Molecular Medicine (MDC), Berlin, Germany.
• PloS one.PLoS One.2013 May 30;8(5):e64888. doi: 10.1371/journal.pone.0064888. Print 2013.
• BACKGROUND: Autoimmune diseases result from a breakdown in self-tolerance to autoantigens. Self-tolerance is induced and sustained by central and peripheral mechanisms intended to deviate harmful immune responses and to maintain homeostasis, where regulatory T cells play a crucial role. The use of s
• PMID 23738007

Japanese Journal

• Increased T-cell immunity against aquaporin-4 and proteolipid protein in neuromyelitis optica.

• Matsuya Nemu,Komori Mika,Nomura Kyouichi,Nakane Shunya,Fukudome Takayasu,Goto Hirofumi,Shiraishi Hirokazu,Wandinger Klaus-Peter,Matsuo Hidenori,Kondo Takayuki
• International Immunology 23(9), 565-573, 2011-09
• … Next, T-cell responses to AQP4 and myelin peptides were studied in 12 NM0 patients, 10 multiple sclerosis (MS) patients and 10 healthy subjects (HS). … Four hours after adding 1 of 28 overlapping AQP4 peptides, a mixture of AQP4 peptides (AQP4-M) or one of six distinct myelin peptides to 2-day cultured PBMC, CD69 expression on CD4+ T cells was examined. …
• NAID 120003751847

• Heat shock protein 70 associations with myelin basic protein and proteolipid protein in multiple sclerosis brains

• CWIKLINSKA Hanna,NYCKO Marcin P.,LUVSANNOROV Otgonbajar,WALKOWIAK Bogdan,BROSNAN Celia F.,RAINE Cedric S.,SELMAJ Krzysztof W.
• International immunology 15(2), 241-249, 2003-02-01
• NAID 10011231013

Related Links

• Proteolipid protein 1 - Wikipedia, the free encyclopedia

This gene encodes a transmembrane proteolipid protein that is the predominant myelin protein present in the central nervous system ... "Molecular diagnostics for myelin proteolipid protein gene mutations in Pelizaeus-Merzbacher disease. ...

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リンク元	「ミエリンプロテオリピドタンパク質」
関連記事	「myel」「proteolipid protein」

「ミエリンプロテオリピドタンパク質」

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英

myelin proteolipid protein

「myel」

　　[★]

• comb form

• 髄、脊髄、骨髄の意味

• ex.

• myeloncephalon 髄脳

「proteolipid protein」

　　[★]

プロテオリピドタンパク質

関

PLP