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torsdag 24 maj 2018

MTMR fosfataasiperhe: . PIKFYVE ja MTMR3 tuottavat PI(5)P

https://www.ncbi.nlm.nih.gov/pubmed/17917119

MTMR FOSFATAASEJA ON ISO PERHE.
(MTMR3 omaa  FYVE domaanitkin . katson sen tunnetut ja suositellut nimet)


 Otan tästä lähdetietoa. ihmiselläkin on niitä 14 proteiinia ja niistä kahdeksan  ovat katalyyttiseti aktiivea fosfataasena  ja kuusi katalyyttisesti inaktiivia.  Aktiiveilla MTMR-jäsenillä on kaikilla  fosfataasiaktiivisuutta  PI3P ja PI(3,5)P2  fosfoinositidilajeja kohtaan.  (Tässä käytetään  uutta termiäkin pylofosfoinositidit, PPIn)  - ja  tämä spesifisyys viittaisi intrasellulaariseen  liikenteeseen ja kalvohomeostaasiin.  MTMR- lyhennys tarkoitta Myotubulariinin liittyvien  fosfolipidien  fosfataasi 

Mol Neurobiol. 2007 Jun;35(3):308-16.
  • Myotubularin-related (MTMR) phospholipid phosphatase proteins in the peripheral nervous system. Bolis A1, Zordan P, Coviello S, Bolino A. Abstract Myotubularin-related proteins (MTMRs) constitute a broad family of ubiquitously expressed phosphatases with 14 members in humans, of which eight are catalytically active phosphatases, while six are catalytically inactive. Active MTMRs possess 3-phosphatase activity toward both PtdIns3P and PtdIns(3, 5)P 2 poliphosphoinositides (PPIn), suggesting an involvement in intracellular trafficking and membrane homeostasis.

Näitten MTMR- fosfataasien joukossa MTMR2 ja MTMR13 eivät  kata toistensa funktioita  hermossa.  Jommankumman puute aiheuttaa CMT tyyppi 4B1 ja B2 neuropatiat vastaavasti. Tyypillistä on  demyelinaatio  ja  poikkeukselliset myeliinipoimut.
MTMR2 fosfolipidifosfataasi sattaa säädellä  kalvohomeostaasissa  taaphtumia Schwannin soluissa  hermon postnataalin kehittymisen aiakna.   Artikkeli valaisee lähinnä MTMR2 ja MTMR13 kannalta  näitä MTMR perheen jsäeniä. solunsisäisiä

  •  Among MTMRs, catalytically active MTMR2 and inactive MTMR13 have a nonredundant function in nerve. Loss of either MTMR2 or MTMR13 causes Charcot-Marie-Tooth type 4B1 and B2 neuropathy, respectively, characterized by demyelination and redundant loops of myelin known as myelin outfoldings. In Mtmr2-null mouse nerves, these aberrant foldings occur at 3-4 weeks after birth, a time when myelination is established, and Schwann cells are still elongating to reach the final internodal length. Moreover, Mtmr2-specific ablation in Schwann cells is both sufficient and necessary to provoke CMT4B1 with myelin outfoldings. MTMR2 phospholipid phosphatase might regulate intracellular trafficking events and membrane homeostasis in Schwann cells during postnatal nerve development. In this review, we will discuss recent findings on the MTMR family with a major focus on MTMR2 and MTMR13 and their putative role in Schwann cell biology.
PMID:
17917119
[Indexed for MEDLINE]
  • MTMR3 on ZFYVE10
https://www.ncbi.nlm.nih.gov/gene/8897
Also known as ZFYVE10; FYVE-DSP1
Summary This gene encodes a member of the myotubularin dual specificity protein phosphatase gene family. The encoded protein is structurally similar to myotubularin but in addition contains a FYVE domain and an N-terminal PH-GRAM domain. The protein can self-associate and also form heteromers with another myotubularin related protein. The protein binds to phosphoinositide lipids through the PH-GRAM domain, and can hydrolyze phosphatidylinositol(3)-phosphate and phosphatidylinositol(3,5)-biphosphate in vitro. The encoded protein has been observed to have a perinuclear, possibly membrane-bound, distribution in cells, but it has also been found free in the cytoplasm. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008] Expression Ubiquitous expression in bone marrow (RPKM 20.3), testis (RPKM 13.1) and 25 other tissues See more Orthologs mouse all

GeneRIFs: Gene References Into FunctionsWhat's a GeneRIF?

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