BCKDHB FISH Probe
The BCKDHB FISH probe is designed to hybridize to the BCKDHB gene and is primarily used for detecting amplifications and deletions associated with the gene. This probe is FISH confirmed on normal peripheral blood metaphase spreads and interphase nuclei. The probe can be labeled in one of five colors. Each probe is sold in a 20 test kit (approximately 20 slides - 22x22 mm area) and includes hybridization buffer. Please note that due to design optimizations, prices are subject to change.
** This product is for in vitro and research use only. This product is not intended for diagnostic use.
| SKU | Test Kits | Buffer | Dye Color | Order Now |
|---|---|---|---|---|
| BCKDHB-20-OR (Standard Design) | 20 (40 μL) | 200 μL |  |
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| BCKDHB-20-RE | 20 (40 μL) | 200 μL |  |
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| BCKDHB-20-GO | 20 (40 μL) | 200 μL |  |
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| BCKDHB-20-GR | 20 (40 μL) | 200 μL |  |
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| BCKDHB-20-AQ | 20 (40 μL) | 200 μL |  |
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| BCKDHB-20-DIG | 20 (40 μL) | 200 μL |  |
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| BCKDHB-20-BIO | 20 (40 μL) | 200 μL |  |
Gene Summary
This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation and feeding problems. Alternative splicing at this locus results in multiple transcript variants. [provided by RefSeq, Jan 2016]
Gene Details
Gene Symbol: BCKDHB
Gene Name: Branched Chain Keto Acid Dehydrogenase E1 Subunit Beta
Chromosome: CHR6: 80816343-81055987
Locus: 6q14.1
FISH Probe Protocols
| Protocol, Procedure, or Form Name | Last Modified | Download |
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Customer Publications
Product Details
Product: BCKDHB FISH Probe
Test Kits: 20 (40 μL)
ISH Buffer: 200 μL
SKU: BCKDHB-20-OR
Material Safety Data Sheet: MSDS.pdf
Turnaround Time: 7-10 Business Days
Shipping Time: 1-2 Day Expedited Shipping