Anti-DHTKD1 Monoclonal Antibody for WB, IF/ICC, ELISA - Q96HY7

Core Product Specifications and Parameters

Biological Background: DHTKD1 Function and Localization

• DHTKD1 encodes the E1a component of the 2-oxoadipate dehydrogenase complex, which catalyzes the irreversible decarboxylation of 2-oxoadipate to glutaryl-CoA. Related references: PMID:29191460, PMID:32695416
• The enzyme prefers 2-oxoadipate as substrate but can also use 2-oxoglutarate at a lower rate in vitro. Related references: PMID:29191460, PMID:29752936, PMID:32633484
• It functions in the final steps of L-lysine, L-hydroxylysine, and L-tryptophan catabolism.
• The protein localizes to the mitochondrion.
• Mutations in DHTKD1 are linked to Charcot-Marie-Tooth disease type 2Q (CMT2Q), a peripheral neuropathy.
• Alternative names include AAKAD, AMOXAD, and OADC-E1.
• Molecular weight is approximately 103 kDa (calculated 103077 Da).

Experimental Guidance and Technical Tips

• The immunogen corresponds to the N-terminal region (aa 32-197) of human DHTKD1; consider validating detection of full-length endogenous protein in your model system.
• For Western blot, optimize antibody dilution using a nuclear/mitochondrial extract or cell line known to express DHTKD1; the expected band size is ~103 kDa.
• For IF/ICC, anticipate a mitochondrial staining pattern; co-staining with a mitochondrial marker is recommended to confirm localization.
• For ELISA, the antibody may be used for direct detection; test pairing with a suitable secondary antibody for sandwich assays.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio is your one-stop sourcing partner for IVD raw materials, providing validated antibody pairs, monoclonal and polyclonal antibodies, bulk ancillary reagents, and rare-target sourcing. For metabolic enzyme targets like DHTKD1, our portfolio supports research into disorders related to amino acid catabolism and neurodegenerative diseases. We help diagnostic manufacturers accelerate development from concept to clinic with reliable supply chain solutions.