BCS1L Rabbit Polyclonal Antibody for WB, IHC-P, IF/ICC, ELISA - Q9Y276

Core Product Specifications and Parameters

Biological Background: BCS1L Function and Localization

• BCS1L, also known as BCS1-like protein, is a mitochondrial chaperone encoded by the BCS1L gene (Gene ID: 617). It belongs to the AAA+ ATPase family and contains an ATP-binding domain.
• It functions as a chaperone necessary for the incorporation of Rieske iron-sulfur protein UQCRFS1 into complex III of the mitochondrial respiratory chain. Related references: PMID:11528392, PMID:9878253
• BCS1L also plays an important role in maintaining mitochondrial tubular networks, respiratory chain assembly, and the formation of the LETM1 complex. Related references: PMID:18628306
• The protein localizes to the mitochondrial inner membrane and possesses transmembrane domains, consistent with its membrane-integrated function.
• It is ubiquitously expressed, highlighting its essential housekeeping role in mitochondrial biology.
• Mutations in BCS1L are associated with severe mitochondrial disorders, including GRACILE syndrome, Björnstad syndrome, and complex III deficiency (MC3DN1), often featuring sensorineural deafness; thus, BCS1L is clinically relevant for mitochondrial disease research.

Experimental Guidance and Technical Tips

• The immunogen consists of a recombinant fusion protein corresponding to amino acids 33–240 of human BCS1L. For western blot (WB), a band near 48 kDa is expected; validate in the appropriate sample lysates, such as mitochondrial fractions or whole cell extracts from human, mouse, or rat cells.
• For immunohistochemistry (IHC‑P), consider using formalin-fixed paraffin-embedded tissue sections with heat-induced antigen retrieval; optimize primary antibody concentration on known positive tissues.
• In immunofluorescence/immunocytochemistry (IF/ICC), include mitochondrial counterstains (e.g., MitoTracker) to confirm co‑localization; permeabilization conditions should be validated for nuclear and mitochondrial membranes.
• This antibody may be suitable for ELISA; optimize coating antigen and detection conditions.
• Cross‑reactivity with mouse and rat suggests utility in preclinical models; however, always verify performance in the species of interest.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio is your one-stop IVD raw-material sourcing partner, offering a comprehensive range of antibodies, antigens, and ancillary reagents for diagnostic assay development. For BCS1L and mitochondrial disease research, we supply validated antibody pairs, bulk quantities of polyclonal and monoclonal antibodies, and rare-target sourcing to support your projects from concept to clinical application.