Avtor/Urednik		Milisav, Irina
Naslov		Translocases of the mitochondrial inner membrane
Prevedeni naslov		Translokaze v notranji mitohondrijski membrani
Tip		članek
Vir		Acta Biol Slov
Vol. in št.		Letnik 44, št. 1-2
Leto izdaje		2001
Obseg		str. 13-23
Jezik		eng
Abstrakt		Mitochondria are organelles enclosed by an outer and an inner membrane. Most mitochondrial proteins are synthesized in the cytoplasm and are transported through the outer and often through the inner membrane. Despite the protein translocation, the proton gradient must be maintained across the inner membrane. To meet this requirement, multicomponent protein translocation systems evolved. While the outer membrane carries only one kind of complex for protein import, there are two different kinds of such complexes in the inner membrane. A recently discovered component of a yeast import complex, TimB, is similar to the human deafness dystonia polypeptide 1, whose mutation gives the neurodegenerative deafness dystonia syndrome. This suggests that a defect in the import mechanism is the cause of this disease. The composition and the function of the mitochondrial protein import complexes are discussed below.
Izvleček		Mitohondriji so organeli obdani z dvema celičnima membranama: z zunanjo in z notranjo membrano. Večina beljakovin, potrebnih za rast in funkcijo, se sintetizira na ribosomih v citoplazmi in mora preiti obe membrani. Navkljub translokaciji beljakovin se mora vzdrževati protonski gradient preko notranje mitohondrijske membrane. Prenos beljakovin v mitohondrije zato opravljajo veliki kompleksi beljakovin, en v zunanji in dva v notranji mitohondrijski membrani. Pred kratkim odkrita komponenta Tim8 kaže na vlogo teh kompleksov pri bolezni, sindromu gluhosti in distonije. V prispevku sta opisani sestava in funkcija kompleksov za vnos beljakovin v mitohondrij.
Deskriptorji		MITOCHONDRIA PROTEINS BIOLOGICAL TRANSPORT, ACTIVE INTRACELLULAR MEMBRANES