DBT Antibody
Description
Available DBT Antibodies
Commercial DBT antibodies vary in reactivity, applications, and validation data. Key options include:
Key Features:
-
Proteintech 12451-1-AP: Validated in HepG2 cells, brain tissues, and cancer samples (e.g., stomach cancer) .
-
Abcam ab151991: Detects recombinant fragments (aa 1–250) with high specificity in WB and ICC/IF .
Research Applications
DBT antibodies are utilized in diverse experimental contexts:
Western Blot (WB)
-
Detects endogenous DBT at ~53 kDa in human, mouse, and rat tissues .
-
Example: Confirmed reduced DBT expression in clear cell renal cell carcinoma (ccRCC) tissues compared to normal samples .
Immunohistochemistry (IHC)
-
Localizes DBT in mitochondrial matrices of neuronal and cancer tissues .
-
Protocol: Antigen retrieval with TE buffer (pH 9.0) or citrate buffer (pH 6.0) .
Immunofluorescence (IF)
Functional Studies
-
Overexpression of DBT in renal cancer cells inhibits proliferation and migration .
-
Loss of DBT activates autophagy via AMPK, mitigating proteotoxicity in neurodegenerative models .
Neurodegenerative Diseases
-
ALS/FTD Pathogenesis: Elevated DBT levels in ALS patient spinal cords correlate with TDP-43 proteinopathy. DBT knockout reduces proteasome inhibition-induced cytotoxicity by enhancing autophagy .
-
Mechanism: DBT depletion increases AMPK activity, promoting ubiquitinated protein clearance .
Cancer Biology
-
Renal Cancer: DBT overexpression suppresses ccRCC aggressiveness by slowing proliferation (CCK-8 assay) and reducing migration (wound healing assay) .
-
Immune Modulation: Low DBT expression in ccRCC correlates with increased immune infiltration (e.g., M1 macrophages, dendritic cells) and poor prognosis .
Metabolic Regulation
-
DBT is a metabolic switch linking BCAA catabolism to proteostasis. Its inhibition redirects cellular energy toward autophagy under proteotoxic stress .
Recommended Dilutions
Validation and Citations
Product Specs
Target Background
- The novel DBT mutation c.650-651insT was more prevalent than the deleted 4.7-kb heterozygote in the Amis population. The reported 4.7-kb deletion, indicating a possible founder mutation, may be preserved. PMID: 24268812
- Deletion in the DBT gene is associated with maple syrup urine disease. PMID: 23313820
- Four novel mutations in the DBT gene resulted in intermittent maple syrup urine disease in seven Norwegian patients. The pathogenic effect of these mutations is the depletion of cellular protein. The intermittent form of MSUD appears to be due to residual R301C mutant protein. PMID: 20570198
- A distinct subset of antimitochondrial antibodies recognizes sequences on branched-chain acyltransferase located outside of the lipoyl binding domain in primary biliary cirrhosis and overlap syndrome with autoimmune hepatitis. PMID: 14768949
- The presence of the interdomain linker restricts the motional freedom of the hbSBD more significantly than hbLBD. The linker region likely exists as a soft rod rather than a flexible string in solution. PMID: 16861235
- In our cohort, more severe enzyme and clinical phenotypes of variant maple syrup urine disease were mainly associated with specific genotypes in the BCKDHA gene. Milder enzyme and clinical phenotypes were associated with specific genotypes in the BCKDHB and DBT genes. PMID: 17922217
- Thirty Portuguese patients with maple syrup urine disease were studied. Seventeen putative mutations were identified (six in BCKDHA, five in BCKDHB, and six in DBT). Seven of these are described for the first time. PMID: 18378174
- Examination of the deletion mutation in the E2 (DBT) gene facilitated early MSUD diagnosis and was beneficial for determining the proper course of treatment. PMID: 18533943
- In 37% (12 patients) of a total of 64 alleles, the supposed maple syrup urine disease-causing mutations in Turkish patients were located in the BCKDHA gene. In 44% (14 patients), mutations were found in the BCKDHB gene, and in 19% (6 patients) in the DBT gene. PMID: 19480318
- Two novel type IB MSUD mutations were identified in Israeli patients. These mutations affect the E1beta subunit in the decarboxylase (E1) component of the branched-chain alpha-ketoacid dehydrogenase complex. PMID: 11448970
- Mutation in the DBT gene causes a subset of maple syrup urine disease in the Ashkenazi Jewish population. PMID: 11509994
