Anti-BCS1L Rabbit pAb
- 100ug (NP31911) Please inquiry
Antibody type:Primary antibody
Label:Unconjugated
Modification:Unmodification
Isotype:Rabbit IgG
Host:Rabbit
Application:WB,IHC,IF
Purify method:Affinity purified
Species:Human,Mouse,Rat
Gene Name:BCS1L
Synonyms:BCS; BJS; PTD; BCS1; FLNMS; h-BCS; MC3DN1; h-BCS1; GRACILE; Hs.6719
Gene Synonyms:
Gene Full Name:BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone
Gene Infomation: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. Several alternatively spliced transcripts encoding two different isoforms have been described. [provided by RefSeq, Jan 2016]
Antigen:Recombinant fusion protein containing a sequence corresponding to amino acids 33-240 of human BCS1L (NP_004319.1).
Antigen Synonyms:Mitochondrial chaperone BCS1
Clonality:Polyclonal antibody
Source:Human
Reaction:
Form:Liquid
Tested Applications:
- Western blot (1:100 to 1:500)
- Immunofluorescence (1:50 to 1:400)
- Immunohistochemistry (1:200 to 1:500)
- Flow cytometry analysis (1:200 to 1:500)
- Enzyme-linked Immunosorbent Assay (1:100-1:5000)
Clone:
Dilution:WB1:500 -1:2000; IHC1:50 -1:200; IF1:50 -1:200(Optimal dilutions should be determined by the end user)
Mole Mass:48kDa
Location:Membrane, Mitochondrion, Mitochondrion inner membrane
Concentration:
Sequence Similarity:
Gene Id:
SwissProt ID:Q9Y276
Unigene:617
Nucleotide Accession:
Tissue specificity:Ubiquitous.
Storage:Store at -20°C. Avoid freeze / thaw cycles.
Buffer condition:PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Background:
Molar Function:Chaperone necessary for the assembly of mitochondrial respiratory chain complex III. Plays an important role in the maintenance of mitochondrial tubular networks, respiratory chain assembly and formation of the LETM1 complex.
Western Blot:Stability: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.
Storage: Store at 4°C for frequent use. Stored at -20°C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.
Notes: For In vitro laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.