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Rabbit Anti-HSP47/FITC Conjugated antibody
background:
This protein is a member of the serpin superfamily of serine proteinase inhibitors. Its expression is induced by heat shock. The protein localizes to the endoplasmic reticulum lumen and binds collagen; thus it is thought to be a molecular chaperone involved in the maturation of collagen molecules. Autoantibodies to this protein have been found in patients with rheumatoid arthritis.
Function:
Binds specifically to collagen. Could be involved as a chaperone in the biosynthetic pathway of collagen.
Subcellular Location:
Endoplasmic reticulum lumen.
DISEASE:
Osteogenesis imperfecta 10 (OI10) [MIM:613848]: A form of osteogenesis imperfecta, a connective tissue disorder characterized by low bone mass, bone fragility and susceptibility to fractures after minimal trauma. Disease severity ranges from very mild forms without fractures to intrauterine fractures and perinatal lethality. Extraskeletal manifestations, which affect a variable number of patients, are dentinogenesis imperfecta, hearing loss, and blue sclerae. OI10 is an autosomal recessive form characterized by multiple bone deformities and fractures, generalized osteopenia, dentinogenesis imperfecta, and blue sclerae. Note=The disease is caused by mutations affecting the gene represented in this entry.
Similarity:
Belongs to the serpin family.
Database links:
Entrez Gene: 510850 Cow
Entrez Gene: 871 Human
Entrez Gene: 1486 Mouse
Entrez Gene: 29345 Rat
Omim: 600943 Human
SwissProt: Q2KJH6 Cow
SwissProt: P50454 Human
SwissProt: P19324 Mouse
SwissProt: P29457 Rat
Unigene: 596449 Human
Unigene: 22708 Mouse
Unigene: 444951 Mouse
Unigene: 98199 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
信号传导(Signaling Intermediates)
HSP47是一类进化上高度保守的蛋白质家族.生理状态时协助多肽或蛋白质的正确转位、折叠和装配,起"分子伴侣"的作用;在应激状态下,HSP47过表达或异位表达,作为一种自身抗原被免疫系统识别,诱发机体的保护性免疫应答,也可作为一种信号分子,在信号转导中发挥作用.
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