PRODH
PRODH (proline dehydrogenase 1, also known as proline oxidase, POX, HSPOX2, PRODH1, PRODH2, PIG6 and TP53I6) is a mitochondrial flavoprotein that catalyses the first step in proline degradation. It converts proline to delta-1-pyrroline-5-carboxylate. Browse Assay Genie's current range of PRODH research reagents below, or read on for the biology behind the target.
PRODH is a mitochondrial enzyme that carries out the first step of proline catabolism, converting proline to delta-1-pyrroline-5-carboxylate. It is located in the mitochondrial matrix. The HGNC places it in the flavoprotein group and the proline dehydrogenase family, and UniProt notes that it belongs to the proline oxidase family. The enzyme is classified as EC 1.5.5.2.
The human PRODH gene encodes the PRODH protein and lies on chromosome 22q11.21. RefSeq reports that alternatively spliced transcript variants encoding different isoforms have been found. The mouse ortholog is Prodh, which is relevant when choosing a model system for work on proline metabolism.
The name has changed over time. The enzyme was previously listed as proline dehydrogenase (proline oxidase), and the aliases POX, HSPOX2, PRODH1, PRODH2, PIG6 and TP53I6 still appear in the literature and in databases. Researchers who search for a PRODH antibody, PRODH ELISA kit or PRODH protein may therefore also meet these older names.
Mutations in the gene are associated with hyperprolinemia type 1 and with susceptibility to schizophrenia 4 (SCZD4). The 22q11.21 region has also been associated with the contiguous gene deletion syndromes DiGeorge and CATCH22. This makes PRODH a useful subject for studies that link amino acid metabolism, mitochondrial biology and disease genetics.
PRODH catalyses the first step in proline degradation, so it is a central enzyme for studying how proline is processed in mitochondria.
As a mitochondrial matrix flavoprotein of the proline oxidase family, PRODH is a model for studying flavin-dependent amino acid oxidation.
Mutations in PRODH are associated with hyperprolinemia type 1, which makes the enzyme a focus for inherited metabolic disorder studies.
PRODH is associated with susceptibility to schizophrenia 4 and lies in the 22q11.21 region linked to DiGeorge and CATCH22 deletion syndromes.
PRODH is a mitochondrial enzyme that catalyses the first step in proline degradation. It converts proline to delta-1-pyrroline-5-carboxylate. It is found in the mitochondrial matrix and belongs to the proline oxidase family of flavoproteins.
PRODH is also called proline dehydrogenase 1 and proline oxidase. Aliases include POX, HSPOX2, PRODH1, PRODH2, PIG6 and TP53I6. RefSeq also reports alternatively spliced transcript variants that encode different isoforms of the protein.
Mutations in PRODH are associated with hyperprolinemia type 1 and susceptibility to schizophrenia 4. The gene sits in the 22q11.21 region linked to DiGeorge and CATCH22 syndromes. The mouse ortholog is Prodh; check the datasheet of any product for its validated reactivity.
Other proline metabolism enzymes and mitochondrial markers may be listed here as further target pages become available.
Gene and protein annotations from HGNC, NCBI Gene and UniProt O43272; product specifications from Assay Genie datasheets.