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BCS1L Monoclonal antibody, PBS Only

BCS1L Monoclonal Antibody for WB,Indirect ELISA,IHC,IF

Host / Isotype

Mouse / IgG1

Reactivity

human, mouse

Applications

WB,Indirect ELISA,IHC,IF

Conjugate

Unconjugated

CloneNo.

3G6H1

Cat No : 60212-1-PBS

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Synonyms

BCS1L, BJS, FLNMS, GRACILE, h BCS, h BCS1, Hs.6719, Mitochondrial chaperone BCS1, PTD



产品信息

60212-1-PBS targets BCS1L in WB, IF, IHC, Indirect ELISA applications and shows reactivity with human, mouse samples.

Tested Applications WB,Indirect ELISA,IHC,IF Application Description
Tested Reactivity human, mouse
Immunogen BCS1L fusion protein Ag18280 种属同源性预测
Host / Isotype Mouse / IgG1
Class Monoclonal
Type Antibody
Full Name BCS1-like (yeast)
Synonyms BCS1L, BJS, FLNMS, GRACILE, h BCS, h BCS1, Hs.6719, Mitochondrial chaperone BCS1, PTD
Calculated Molecular Weight 48 kDa
Observed Molecular Weight 47 kDa
GenBank Accession NumberBC007500
Gene Symbol BCS1L
Gene ID (NCBI) 617
Conjugate Unconjugated
Form Liquid
Purification MethodProtein G purification
UNIPROT IDQ9Y276
Storage Buffer PBS Only
Storage ConditionsStore at -80°C.

背景介绍

Human BCS1-like (BCS1L), a mitochondrial inner-membrane protein, is a chaperone necessary for the assembly of mitochondrial respiratory chain complex III. Recently studies indicated that the mutations in this protein may cause the GRACILE (growth retardation, aminoaciduria, cholestasis, iron overload, lactacidosis, and early death) syndrome, a recessively inherited lethal disease characterized by fetal growth retardation, lactic acidosis, aminoaciduria, cholestasis, and abnormalities in iron metabolism.