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BCS1L, Polyclonal Antibody

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产品名称: BCS1L, Polyclonal Antibody
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简单介绍

BCS1L, Polyclonal Antibody


BCS1L, Polyclonal Antibody  的详细介绍
Product Name

BCS1L, Polyclonal Antibody

Popular Item
Full Product Name

BCS1L Antibody

Product Synonym Names
BCS 1; BCS 1L; BCS; BCS1 (yeast homolog) like; BCS1; BCS1 like (S. cerevisiae); BCS1 like; BCS1 like protein; Bjornstad syndrome (pili torti and deafness); BJS; FLNMS; GRACILE; h BCS; H BCS1; Hs.6719; Mitochondrial chaperone BCS1; Mitochondrial complex III assembly; PTD
Product Gene Name

anti-BCS1L antibody

[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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3D Structure
ModBase 3D Structure for Q9Y276
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human, Mouse, Rat
Specificity
BCS1L antibody detects endogenous levels of BCS1L.
Purity/Purification
The antiserum was purified by peptide affinity chromatography using SulfoLink Coupling Resin.
Form/Format
Liquid
Phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Concentration
1mg/ml (lot specific)
Immunogen
A synthesized peptide derived from human BCS1L.
Subcellular Location
Mitochondrial. Mitochondrion inner membrane. Single-pass membrane protein.
Tissue Specificity
Ubiquitous.
Predicted Cross Reactivity
Pig, Bovine, Horse, Sheep, Rabbit, Dog
Similarity
Pig (100%), Bovine (100%), Horse (100%), Sheep (100%), Rabbit (92%), Dog (100%)
Conjugation
Unconjugated
Preparation and Storage
Store at -20 degree C. Stable for 12 months from date of receipt.
ISO Certification
Manufactured in an ISO 9001:2015 Certified Laboratory.
Supply Chain Verification
Manufactured in a lab with traceable raw materials manufactured on site. Coordinated product portfolio of antibodies, pairs, conjugates, recombinant proteins, and immunoassay materials available, please inquire.
Other Notes
Small volumes of anti-BCS1L antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Related Product Information for
anti-BCS1L antibody
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.
Subunit Structure: Interacts with LETM1.
Similarity: Belongs to the AAA ATPase family. BCS1 subfamily.
Applications Tested/Suitable for anti-BCS1L antibody
Western Blot (WB), Immunohistochemistry (IHC)
Application Notes for anti-BCS1L antibody
WB: 1:500-1:2000
IHC: 1:50-1:200

Western Blot (WB) of anti-BCS1L antibody
Western blot analysis of extracts from HepG2, using BCS1L antibody. Lane 1 was treated with the blocking peptide.
anti-BCS1L antibody Western Blot (WB) (WB) image
Immunohistochemistry (IHC) of anti-BCS1L antibody
MBS9603417 at 1/100 staining Human breast cancer tissue by IHC-P. The sample was formaldehyde fixed and a heat mediated antigen retrieval step in citrate buffer was performed. The sample was then blocked and incubated with the antibody for 1.5 hours at 22 degree C. An HRP conjugated goat anti-rabbit antibody was used as the secondary.
anti-BCS1L antibody Immunohistochemistry (IHC) (IHC) image
Western Blot (WB) of anti-BCS1L antibody
Western blot analysis of extracts of HEK-293 cells, using BCS1L antibody.
anti-BCS1L antibody Western Blot (WB) (WB) image
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NCBI/Uniprot data below describe general gene information for BCS1L. It may not necessarily be applicable to this product.
NCBI GI #
119964730
NCBI GeneID
617
NCBI Accession #
NP_001073335.1 [Other Products]
NCBI GenBank Nucleotide #
NM_001079866.1 [Other Products]
UniProt Primary Accession #
Q9Y276 [Other Products]
UniProt Secondary Accession #
Q7Z2V7; B3KTW9[Other Products]
UniProt Related Accession #
Q9Y276[Other Products]
Molecular Weight
Observed: 50-55 kDa
Predicted: 48 kDa
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NCBI Official Full Name
mitochondrial chaperone BCS1 isoform a
NCBI Official Synonym Full Names
BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone
NCBI Official Symbol
BCS1L  [Similar Products]
NCBI Official Synonym Symbols
BCS; BJS; PTD; BCS1; FLNMS; h-BCS; MC3DN1; h-BCS1; GRACILE; Hs.6719
  [Similar Products]
NCBI Protein Information
mitochondrial chaperone BCS1
UniProt Protein Name
Mitochondrial chaperone BCS1
UniProt Synonym Protein Names
BCS1-like protein
UniProt Gene Name
BCS1L  [Similar Products]
UniProt Synonym Gene Names
BCS1; h-BCS1  [Similar Products]
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NCBI Summary for BCS1L
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]
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UniProt Comments for BCS1L
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.
Research Articles on BCS1L
1. The BCSIL gene mutation is responsible for GRACILE syndrome, Bjornstad syndrome and complex III deficiency. Bjomstad syndrome is characterized by sensorineural hearing loss and abnormal flat twisted hair shafts. The case is GRACILE syndrome with Bjomstad phenotype in neonatal period due to BCSL1 gene mutation.
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Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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