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Anti-SERPINH1 Antibody

品牌：Antibodies

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规格：50µl

货期：

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Anti-SERPINH1 Antibody

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Name:	Anti-SERPINH1 Antibody See all SERPINH1 primary antibodies
Description:	Rabbit polyclonal antibody to SERPINH1.
Applications:	WB, IHC, IF
Dilutions:	WB: 1:500 - 1:2000, IHC: 1:50 - 1:200, IF: 1:50 - 1:200.
Reactivity:	Human, Mouse, Rat
Immunogen:	Recombinant protein of human SERPINH1.
Protein Length:	418
Host:	Rabbit
Clonality:	Polyclonal
Isotype:	IgG
Conjugate:	Unconjugated
Purification:	Affinity purification.
Product Form:	Liquid
Formulation:	Supplied in Phosphate Buffered Saline, pH 7.30, with 0.02% Sodium Azide and 50% Glycerol.
Storage:	Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

Function:	Binds specifically to collagen. Could be involved as a chaperone in the biosynthetic pathway of collagen.
Involvement in Disease:	Osteogenesis imperfecta 10: 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.
Sequence Similarities:	Belongs to the serpin family.
Cellular Location:	Endoplasmic reticulum lumen.