BCS1L Antibody (5F3) - Azide and BSA Free

Western Blot: BCS1L Antibody (5F3) [H00000617-M01] - 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.

• Reactivity: Hu
• Applications: WB, ELISA
• Clone: 5F3
• Clonality: Monoclonal
• Host: Mouse
• Conjugate: Unconjugated
• Format: Azide and BSA Free

BCS1L Antibody (5F3) - Azide and BSA Free Summary

• Description: Quality control test: Antibody Reactive Against Recombinant Protein.
• Immunogen: BCS1L (NP_004319, 320 a.a. ~ 418 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. ASTEARIVFMTTNHVDRLDPALIRPGRVDLKEYVGYCSHWQLTQMFQRFYPGQAPSLAENFAEHVLRATNQISPAQVQGYFMLYKNDPVGAIHNAESLR
• Specificity: BCS1L - BCS1-like (yeast)
• Isotype: IgG1 Kappa
• Clonality: Monoclonal
• Host: Mouse
• Gene: BCS1L
• Purity: IgG purified

Applications/Dilutions

• Dilutions:
  • ELISA
  • Western Blot 1:500
• Application Notes: Antibody reactivity against transfected lysate and recombinant protein for WB. It has been used for ELISA.

Packaging, Storage & Formulations

• Storage: Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles.
• Buffer: In 1x PBS, pH 7.4
• Preservative: No Preservative
• Purity: IgG purified

Notes

This product is produced by and distributed for Abnova, a company based in Taiwan.

Alternate Names for BCS1L Antibody (5F3) - Azide and BSA Free

• BCS
• BCS1 (yeast homolog)-like
• BCS1
• BCS1-like (S. cerevisiae)
• BCS1-like (yeast)
• BCS1-like protein
• BJSGRACILE
• FLNMS
• h-BCS
• h-BCS1
• mitochondrial chaperone BCS1
• mitochondrial complex III assembly
• PTD

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.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Publications for BCS1L Antibody (H00000617-M01)(4)

Publications using H00000617-M01

• Mirali S, Botham A, Voisin V et al. The mitochondrial peptidase, neurolysin, regulates respiratory chain supercomplex formation and is necessary for AML viability. Sci Transl Med. 2020-04-08 [PMID: 32269163]
• Olahova M, Berti C, Collier J et al. Molecular genetic investigations identify new clinical phenotypes associated with BCS1L-related mitochondrial disease. Hum Mol Genet. 2019-08-22 [PMID: 31435670]
• Kotarsky H, Karikoski R, Morgelin M et al. Characterization of complex III deficiency and liver dysfunction in GRACILE syndrome caused by a BCS1L mutation. Mitochondrion. 2010-01-01 [PMID: 20580947]
• Gil-Borlado MC, Gonzalez-Hoyuela M, Blazquez A et al. Pathogenic mutations in the 5' untranslated region of BCS1L mRNA in mitochondrial complex III deficiency. Mitochondrion. 2009-04-21 [PMID: 19389488]

Bioinformatics

• Gene Symbol: BCS1L
• Entrez:
  • 617(Human)
• OMIM:
  • 124000(Human)
• Uniprot:
  • NP_004319(Human)