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LIMP-2/SCARB2  Protein, Antibody, ELISA Kit, cDNA Clone

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表達宿主: Human Cells  
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11063-H03H-50
11063-H03H-100
50 µg 
100 µg 
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表達宿主: Human Cells  
  • Slide 1
50052-M08H-50
50052-M08H-100
50 µg 
100 µg 
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LIMP-2/SCARB2 相關信號通路

LIMP-2/SCARB2 概述&蛋白信息

LIMP-2/SCARB2 研究背景

基因概述: The protein encoded by this SCARB2 gene is a type III glycoprotein that is located primarily in limiting membranes of lysosomes and endosomes. Earlier studies in mice and rat suggested that this protein may participate in membrane transportation and the reorganization of endosomal/lysosomal compartment. LIMP-2 deficiency in mice was reported to impair cell membrane transport processes and cause pelvic junction obstruction, deafness, and peripheral neuropathy. Further studies in human showed that this protein is a ubiquitously expressed protein and that it is involved in the pathogenesis of HFMD (hand, foot, and mouth disease) caused by enterovirus-71 and possibly by coxsackievirus A16. Mutations in this SCARB2 gene caused an autosomal recessive progressive myoclonic epilepsy-4 (EPM4), also known as action myoclonus-renal failure syndrome (AMRF). Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Feb 2011]
General information above from NCBI
亞單位結構: Interacts with GBA (via the coiled-coil domain).
亞細胞定位: Lysosome membrane; Multi-pass membrane protein.
相關疾病 : Epilepsy, progressive myoclonic 4, with or without renal failure (EPM4) [MIM:254900]: An autosomal recessive progressive myoclonic epilepsy associated with renal failure in some cases. Cognitive function is preserved. Myoclonus is a brief, involuntary twitching of a muscle or a group of muscles. Note=The disease is caused by mutations affecting the gene represented in this entry.
Note=Genetic variants in SCARB2 can act as modifier of the phenotypic expression and severity of Gaucher disease.
相似的序列: Belongs to the CD36 family.C
General information above from UniProt

Lysosomal Integral Membrane Protein II (LIMPII), also known as SCARB2, LPG85, and CD36L2, is a type I II multi-pass membrane glycoprotein that is located primarily in limiting membranes of lysosomes and endosomes on all tissues and cell types so far examined. This protein may participate in membrane transportation and the reorganization of endosomal/lysosomal compartment. LIMPII is identified as a receptor for EV71 (human enterovirus species A, Enterovirus 71) and CVA16 (coxsackievirus A16) which are most frequently associated with hand, foot and mouth disease (HFMD). Expression of human LIMPII enables normally unsusceptible cell lines to support the viruses’ propagation and develop cytopathic effects. In addition, LIMPII also has been shown to bind thrombospondin-1, may contribute to the pro-adhesive changes of activated platelets during coagulation, and inflammation. Deficiency of the protein in mice impairs cell membrane transport processes and causes pelvic junction obstruction, deafness, and peripheral neuropathy.

LIMP-2/SCARB2 別稱

AMRF,EPM4,LGP85,CD36L2,HLGP85,LIMP-2,LIMPII,SR-BII, [homo-sapiens]
AMRF,CD36L2,HLGP85,LIMP-2,LIMPII,SCARB2,SR-BII, [human]
9330185J12Rik,Cd36l2,LGP85,LIMP-2,MLGP85,Scarb2, [mouse]
LGP85,Cd36l2,LIMP-2,MLGP85,9330185J12Rik, [mus-musculus]

LIMP-2/SCARB2 相關文獻

  • Crombie, R. et al., 1998, J. Biol. Chem. 273: 4855-4863.
  • Febbraio, M. et al., 2001, J. Clin. Invest. 108: 785-791.
  • Kuronita, T. et al., 2002, J. Cell Sci. 115: 4117-4131.
  • Gamp, A.C. et al., 2003, Human Molecular Genetics. 12: 631-646.
  • Eskelinen, E.L. et al., 2003, Trends in Cell Biology. 13: 137-145.
  • Mulcahy, J.V. et al.,2004, Biochem. J. 377 (Pt 3): 741–747.
  • Yamayoshi, S. et al., 2009, Nat Med. 15 (7): 798-801.
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