BCS1L sirna

BCS1L siRNA (Human)

Cat. Num. AAA8241346

• Gene Names: BCS1L; BCS; BJS; PTD; BCS1; FLNMS; h-BCS; MC3DN1; GRACILE; Hs.6719
• Reactivity: Human
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: BCS1L; BCS1L siRNA (Human); BCS1; Mitochondrial chaperone BCS1; h-BCS1; BCS1-like protein; BCS1L sirna

• Host: Synthetic
• Reactivity: Human
• Specificity: BCS1L siRNA (Human) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
• Purity/Purification: > 97%
• Form/Format: Lyophilized powder
• Sequence Length: 419

• Applicable Applications for BCS1L sirna: RNA Interference (RNAi)
• Quality Control: Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
• Directions for Use: We recommends transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
• Components: We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of human BCS1L gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
• Preparation and Storage: Shipped at 4 degree C. Store at -20 degree C for one year.

Related Product Information for BCS1L sirna

siRNA to inhibit BCS1L expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 119964730
• NCBI GeneID: 617
• NCBI Accession #: NP_001073335.1
• NCBI GenBank Nucleotide #: NM_001079866.1
• UniProt Accession #: Q9Y276; Q7Z2V7; B3KTW9
• Molecular Weight: 47,534 Da
• NCBI Official Full Name: mitochondrial chaperone BCS1
• NCBI Official Synonym Full Names: BC1 (ubiquinol-cytochrome c reductase) synthesis-like
• NCBI Official Symbol: BCS1L
• NCBI Official Synonym Symbols: BCS; BJS; PTD; BCS1; FLNMS; h-BCS; MC3DN1; GRACILE; Hs.6719
• NCBI Protein Information: mitochondrial chaperone BCS1
• UniProt Protein Name: Mitochondrial chaperone BCS1
• UniProt Gene Name: BCS1L
• UniProt Synonym Gene Names: BCS1; h-BCS1
• UniProt Entry Name: BCS1_HUMAN

NCBI Description

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. Five alternatively spliced transcripts encoding the same protein have been described. [provided by RefSeq, Mar 2012]

Uniprot Description

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. Defects in BCS1L are the cause of GRACILE syndrome (GRACILE). GRACILE stands for 'growth retardation, aminoaciduria, cholestasis, iron overload, lactic acidosis, and early death'. It is a recessively inherited lethal disease characterized by fetal growth retardation, lactic acidosis, aminoaciduria, cholestasis, and abnormalities in iron metabolism. Defects in BCS1L are a cause of mitochondrial complex III deficiency (MT-C3D). A disorder of the mitochondrial respiratory chain resulting in a highly variable phenotype depending on which tissues are affected. Clinical features include mitochondrial encephalopathy, psychomotor retardation, ataxia, severe failure to thrive, liver dysfunction, renal tubulopathy, muscle weakness and exercise intolerance. Defects in BCS1L are the cause of Bjoernstad syndrome (BJS). BJS is an autosomal recessive condition characterized by sensorineural hearing loss and pili torti. The hearing loss in BJS is congenital and of variable severity. Pili torti (twisted hairs), a condition in which the hair shafts are flattened at irregular intervals and twisted 180 degrees from the normal axis, making the hair extremely brittle, is usually recognized early in childhood. Belongs to the AAA ATPase family. BCS1 subfamily.

Protein type: Chaperone; Membrane protein, integral; Mitochondrial

Chromosomal Location of Human Ortholog: 2q33

Cellular Component: mitochondrion; mitochondrial respiratory chain complex III

Molecular Function: protein binding; ATP binding

Biological Process: mitochondrion organization and biogenesis; mitochondrial respiratory chain complex I assembly; mitochondrial respiratory chain complex IV assembly

Disease: Leigh Syndrome; Gracile Syndrome; Bjornstad Syndrome; Mitochondrial Complex Iii Deficiency, Nuclear Type 1