Protein Domain : LETM1-like, ribosome-binding domain IPR033122

Type  Domain
Description  Mitochondrial ribosomes synthesise core subunits of the inner membrane respiratory chain complexes. In mitochondria, ribosomes are mainly membraneassociated and translation is tightly coupled to the inner mitochondrial membrane. Mdm38/Letm1 is a conserved membrane receptor for mitochondrialribosomes and specifically involved in respiratory chain biogenesis.These proteins play a role in potassium and hydrogen ion exchange [ , ]. Some features found in LETM1, such as a transmembrane domain and a CK2 and PKC phosphorylation site [], are relatively conserved throughout the family. Deletion of LETM1 is thought to be involved in the development of Wolf-Hirschhorn syndrome in humans [].This entry represents the Letm1 ribosome-binding domain (RBD) of Letm1 from animals and MDM38 from yeast. This domain is necessary and sufficient for interaction with mitochondrial ribosomes. It exhibits a predominantly α-helical compact fold and contains extended, highly chargedsurface patches. The conserved regions most probably represent interaction sites for ribosomal RNA or protein subunits, or for proteins involved inmitochondrial protein biogenesis [ , ].
Short Name  LETM1-like_RBD

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