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>   首页   >   产品   >   一抗   >   代谢   >   BCS1L Antibody (monoclonal) (M01)   

BCS1L Antibody (monoclonal) (M01)

Mouse monoclonal antibody raised against a partial recombinant BCS1L.

     
  • 1 - BCS1L Antibody (monoclonal) (M01) AT1287a
    Antibody Reactive Against Recombinant Protein.Western Blot detection against Immunogen (36.63 KDa) .
  • 1 - BCS1L Antibody (monoclonal) (M01) AT1287a
    Western Blot analysis of BCS1L expression in transfected 293T cell line by BCS1L monoclonal antibody (M01), clone 5F3.

    Lane 1: BCS1L transfected lysate(47.534 KDa).
    Lane 2: Non-transfected lysate.
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB
Primary Accession Q9Y276
Other Accession NM_004328
Reactivity Human
Host mouse
Clonality monoclonal
Isotype IgG1 Kappa
Clone Names 5F3
Calculated MW 47534 Da
Additional Information
Gene ID 617
Other Names Mitochondrial chaperone BCS1, h-BCS1, BCS1-like protein, BCS1L, BCS1
Target/Specificity BCS1L (NP_004319, 320 a.a. ~ 418 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Dilution WB~~1:500~1000
Format Clear, colorless solution in phosphate buffered saline, pH 7.2 .
StorageStore at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
PrecautionsBCS1L Antibody (monoclonal) (M01) is for research use only and not for use in diagnostic or therapeutic procedures.
Research Areas

For Research Use Only. Not For Use In Diagnostic Procedures.

BACKGROUND

This gene encodes a homolog of the S. cerevisiae bcs1 protein which is involved in the assembly of complex III of the mitochondrial respiratory chain. The encoded protein does not contain a mitochondrial targeting sequence but experimental studies confirm that it is imported into mitochondria. Mutations in this gene are associated with mitochondrial complex III deficiency and the GRACILE syndrome. Two alternatively spliced transcripts encoding the same protein have been described.

REFERENCES

1.Characterization of complex III deficiency and liver dysfunction in GRACILE syndrome caused by a BCS1L mutation.Kotarsky H, Karikoski R, Morgelin M, Marjavaara S, Bergman P, Zhang DL, Smet J, van Coster R, Fellman F.Mitochondrion (2010), doi:10.1016/ j.mito.2010.05.0092.Pathogenic mutations in the 5' untranslated region of BCS1L mRNA in mitochondrial complex III deficiency.Gil-Borlado MC, Gonzalez-Hoyuela M, Blazquez A, Garcia-Silva MT, Gabaldon T, Manzanares J, Vara J, Martin MA, Seneca S, Arenas J, Ugalde C.Mitochondrion. 2009 Sep;9(5):299-305. Epub 2009 Apr 21.

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