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SUMMARY:The family of mitochondrial transport proteins - Edmund Kunji\, Th
 e Medical Research Council\, Mitochondrial Biology Unit
DTSTART:20111117T143000Z
DTEND:20111117T153000Z
UID:TALK34212@talks.cam.ac.uk
CONTACT:Becky Baglow
DESCRIPTION:Mitochondrial transport proteins translocate nucleotides\, ami
 no acids\, inorganic ions\, fatty acids\, keto acids and cofactors across 
 the inner membrane of mitochondria. These transport steps are required for
  the generation of ATP from the oxidation of sugars and fat\; the breakdow
 n\, synthesis and inter-conversion of amino acids\; the synthesis of haem 
 and iron sulphur clusters\; heat production\; and macromolecular synthesis
  in the mitochondrion. Dysfunctional transport proteins are associated wit
 h rare but severe human diseases\, such as metabolic disorders and muscula
 r and neurodegenerative diseases. The transport proteins share several seq
 uence features\, including a signature motif PX[DE]XX[RK] and three homolo
 gous amino acid sequence repeats. Their structural folds consist of six tr
 ansmembrane -helices and three matrix -helices that are arranged wit
 h threefold pseudo-symmetry in agreement with the sequence repeats. The ce
 ntral cavity contains a single substrate binding site flanked by two conse
 rved and symmetrical triplets of positively and negatively charged residue
 s that have the propensity to form salt bridge networks. During the transp
 ort cycle the disruption and formation of two salt bridge networks are cou
 pled to the opening and closing of the transport protein on either side of
  the mitochondrial inner membrane in an alternating fashion.
LOCATION:Part II room\,  Department of Genetics
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