Mitochondrial Chaperone BCS1 (BCS1L) Antibody
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Description
BCS1L Antibody is a Rabbit Polyclonal Antibody against BCS1L.
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Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Mitochondrial Chaperone BCS1 (BCS1L) |
| Host | Rabbit |
| Reactivity | Human, Mouse, Rat |
| Recommended Dilution | IHC-P: 1/50 - 1/200, IF/ICC: 1/50 - 1/200. Not tested in IHC-F. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Purification | Purified by affinity chromatography. |
| Size 1 | 60 µl |
| Size 2 | 120 µl |
| Size 3 | 200 µl |
| Form | Liquid |
| Tested Applications | IHC, IF/ICC |
| Buffer | PBS, pH 7.3, containing 0.02% sodium azide, 50% glycerol. |
| Availability | Shipped within 5-10 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | Q9Y276 |
| Gene ID | 617 |
| NCBI Accession | NP_004319.1 |
| Background | Antibody anti-BCS1L |
| Status | RUO |
| Note | Concentration: 1 mg/ml - |
Descripción
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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.
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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.
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