SLC25A11

The oxoglutarate/malate carrier transports 2-oxoglutarate across the inner membranes of mitochondria in an electroneutral exchange for malate or other dicarboxylic acids (summary by Iacobazzi et al., 1992 [PubMed 1457818]).

Full Name

solute carrier family 25 (mitochondrial carrier; oxoglutarate carrier), member 11

Function

Catalyzes the transport of 2-oxoglutarate (alpha-oxoglutarate) across the inner mitochondrial membrane in an electroneutral exchange for malate. Can also exchange 2-oxoglutarate for other dicarboxylic acids such as malonate, succinate, maleate and oxaloacetate, although with lower affinity. Contributes to several metabolic processes, including the malate-aspartate shuttle, the oxoglutarate/isocitrate shuttle, in gluconeogenesis from lactate, and in nitrogen metabolism (By similarity).
Maintains mitochondrial fusion and fission events, and the organization and morphology of cristae (PubMed:21448454).
Involved in the regulation of apoptosis (By similarity).
Helps protect from cytotoxic-induced apoptosis by modulating glutathione levels in mitochondria (By similarity).

Biological Process

Biological Process malate transmembrane transportManual Assertion Based On ExperimentIBA:GO_Central
Biological Process oxaloacetate transportManual Assertion Based On ExperimentIBA:GO_Central
Biological Process phosphate ion transmembrane transportManual Assertion Based On ExperimentIBA:GO_Central
Biological Process succinate transmembrane transportManual Assertion Based On ExperimentIBA:GO_Central
Biological Process sulfate transportManual Assertion Based On ExperimentIBA:GO_Central
Biological Process thiosulfate transportManual Assertion Based On ExperimentIBA:GO_Central

Cellular Location

Mitochondrion inner membrane

Involvement in disease

Paragangliomas 6 (PGL6):
An autosomal dominant tumor predisposition syndrome characterized by adult-onset development of paragangliomas, neural crest tumors usually derived from the chromoreceptor tissue of a paraganglion. Paragangliomas can develop at various body sites, including the head, neck, thorax and abdomen. Some of the tumors may secrete biologically active normetanephrine, resulting in secondary hypertension. PGL6 patients are at high risk for metastatic disease.