Results 21 to 30 of about 1,199,861 (319)

Ubiquitin-proteasome dependent mitochondrial protein quality control [PDF]

, 2014
Dysfunctional mitochondria cause many neurodegenerative disorders and with aging in general, mechanisms of mitochondrial quality control are essential for cellular function.
Benischke, Anne-Sophie
core   +1 more source

Neurodegenerative stress related mitochondrial proteostasis [PDF]

, 2015
1.1 Background: Mitochondria are the main site of energy production in most cells. Furthermore, they are involved in a multitude of other essential cellular processes, such as regulating the cellular calcium pool, lipid metabolism and programmed cell ...
Fang, Lei
core   +1 more source

Hsp70 in mitochondrial biogenesis [PDF]

, 1994
The family of hsp70 (70 kilodalton heat shock protein) molecular chaperones plays an essential and diverse role in cellular physiology, Hsp70 proteins appear to elicit their effects by interacting with polypeptides that present domains which exhibit non ...
Stuart, Rosemary A., Neupert, Walter, Cyr, Douglas M.   +2 more
core   +1 more source

The accessory subunit of mitochondrial DNA polymerase {gamma} determines the DNA content of mitochondrial nucleoids in human cultured cells [PDF]

, 2009
The accessory subunit of mitochondrial DNA polymerase γ, POLGβ, functions as a processivity factor in vitro. Here we show POLGβ has additional roles in mitochondrial DNA metabolism.
H. Sembongi, T. Yasukawa, Yasukawa, T, Di Re, M., I. J. Holt, L. J. Bailey, Fersht, A.R., Boyd-Kirkup, JD, Martinsson, P., Holt, I.J., J. N. Spelbrink, Sembongi, H, Spelbrink, JN, Bailey, LJ, Sembongi, H., Goffart, S, P. Martinsson, A. R. Fersht, Di Re, M, Martinsson, P, Reyes, A., J. D. Boyd-kirkup, S. Goffart, Bailey, L.J., Fersht, AR, Wong, TS, T. S. Wong, Reyes, A, M. Di Re, Holt, IJ, J. He, He, J., Goffart, S., A. Reyes, Boyd-Kirkup, J.D., Wong, T.S., He, J, Yasukawa, T., Spelbrink, J.N.   +38 more
core   +1 more source

A morphological view on mitochondrial protein targeting [PDF]

, 1994
Mitochondrial protein targeting includes both intramitochondrial sorting of proteins encoded by the organellar genome and import and subsequent sorting of nuclear encoded precursor proteins. Only a few proteins are encoded by the mitochondrial genome and
Ida J. van der Klei, Walter Neupert, Neupert, Walter, Marten Veenhuis, Veenhuis, Marten, Klei, Ida J. van der   +5 more
core   +1 more source

Protein translocation across mitochondrial membranes [PDF]

, 1992
Protein translocation across biological membranes is of fundamental importance for the biogenesis of organelles and in protein secretion. We will give an overview of the recent achievements in the understanding of protein translocation across ...
Walter Neupert, Ulla Wienhues, Neupert, Walter, Wienhues, Ulla   +3 more
core   +1 more source

MR-1S Interacts with PET100 and PET117 in Module-Based Assembly of Human Cytochrome c Oxidase

Cell Reports, 2017
The biogenesis of human cytochrome c oxidase (COX) is an intricate process in which three mitochondrial DNA (mtDNA)-encoded core subunits are assembled in a coordinated way with at least 11 nucleus-encoded subunits.
Sara Vidoni, Michael E. Harbour, Sergio Guerrero-Castillo, Alba Signes, Shujing Ding, Ian M. Fearnley, Robert W. Taylor, Valeria Tiranti, Susanne Arnold, Erika Fernandez-Vizarra, Massimo Zeviani   +10 more
doaj   +1 more source

A novel mouse model of mitochondrial disease exhibits juvenile-onset severe neurological impairment due to parvalbumin cell mitochondrial dysfunction

Communications Biology, 2023
Mitochondrial diseases comprise a common group of neurometabolic disorders resulting from OXPHOS defects, that may manifest with neurological impairments, for which there are currently no disease-modifying therapies.
Elizaveta A. Olkhova, Carla Bradshaw, Alasdair Blain, Debora Alvim, Doug M. Turnbull, Fiona E. N. LeBeau, Yi Shiau Ng, Gráinne S. Gorman, Nichola Z. Lax   +8 more
doaj   +1 more source

Rapid enrichment of mitochondria from mammalian cell cultures using digitonin

MethodsX, 2021
We describe here a simple method to enrich mitochondrial fractions from mammalian cells for downstream analyses in the lab. Mitochondria purification involves cell lysis followed by separation of the organelles from the rest of the cellular components ...
Bhavna Dixit, Shon Vanhoozer, Nana Abena Anti, Matthew S. O'Connor, Amutha Boominathan   +4 more
doaj   +1 more source

The Mitochondrial Permeability Transition in Mitochondrial Disorders [PDF]

Oxidative Medicine and Cellular Longevity, 2019
Mitochondrial permeability transition pore (PTP), a (patho)physiological phenomenon discovered over 40 years ago, is still not completely understood. PTP activation results in a formation of a nonspecific channel within the inner mitochondrial membrane with an exclusion size of 1.5 kDa.
Justina Šileikytė, Michael Forte
openaire   +2 more sources