Results 111 to 120 of about 470,683 (177)

Cardiolipin deficiency disrupts CoQ redox state and induces steatohepatitis

Metabolic dysfunction-associated steatotic liver disease (MASLD) is a progressive disorder marked by lipid accumulation, leading to steatohepatitis (MASH). A key feature of the transition to MASH involves oxidative stress resulting from defects in mitochondrial oxidative phosphorylation (OXPHOS). Here, we show that pathological alterations in the lipid
Brothwell, Marisa J., Cao, Guoshen, Maschek, J. Alan, Poss, Annelise M., Peterlin, Alek D., Wang, Liping, Baker, Talia B., Shahtout, Justin L., Siripoksup, Piyarat, Pearce, Quentinn J., Johnson, Jordan M., Finger, Fabian M., Prola, Alexandre, Pellizzari, Sarah A., Hale, Gillian L., Manuel, Allison M., Watanabe, Shinya, Miranda, Edwin R., Affolter, Kajsa E., Tippetts, Trevor S., Nikolova, Linda S., Choi, Ran Hee, Decker, Stephen T., Patil, Mallikarjun, Catrow, J. Leon, Holland, William L., Nowinski, Sara M., Lark, Daniel S., Fisher-Wellman, Kelsey H., Mimche, Patrice N., Evason, Kimberley J., Cox, James E., Summers, Scott A., Gerhart-Hines, Zach, Funai, Katsuhiko   +34 more
openaire   +2 more sources

Isolated succinate-CoQ reductase deficiency [PDF]

, 2020
openaire   +1 more source

Pathological and therapeutic mechanisms in CoQ deficiency: the role of the proteins involved in the Q-Junction

, 2023
Mitochondria are present in most cell types. Structurally, mitochondria have two phospholipid membranes that define four biochemically distinct compartments: the outer membrane, the intermembrane space, the inner membrane and the mitochondrial matrix.
openaire   +1 more source

Vps1 and select autophagic machinery are required for coenzyme Q uptake and trafficking to mitochondria. [PDF]

J Biol Chem
Guile MD, Anderson KA, Jain A, Toulmay A, Prinz WA, Clarke CF.   +5 more
europepmc   +1 more source

Cardiolipin deficiency disrupts CoQ-complex III interface in steatohepatitis

Abstract Metabolic dysfunction-associated steatotic liver disease (MASLD) is a progressive disorder marked by lipid accumulation, leading to steatohepatitis (MASH). A key feature of the transition to MASH involves oxidative stress resulting from defects in mitochondrial oxidative phosphorylation (OXPHOS).
Brothwell, Marisa, Cao, Guoshen, Maschek, J. Alan, Poss, Annelise, Peterlin, Alek, Wang, Liping, Baker, Talia, Shahtout, Justin, Siripoksup, Piyarat, Pearce, Quentinn, Johnson, Jordan, Finger, Fabian, Prola, Alexandre, Pellizzari, Sarah, Hale, Gillian, Manuel, Allison, Watanabe, Shinya, Miranda, Edwin, Affolter, Kajsa, Tippetts, Trevor, Nikolova, Linda, Choi, Ran, Decker, Stephen, Patil, Mallikarjun, Catrow, J. Leon, Holland, William, Nowinski, Sara, Lark, Daniel, Fisher-Wellman, Kelsey, Mimche, Patrice, Evason, Kimberley, Cox, James, Summers, Scott, Gerhart-Hines, Zach, Funai, Katsuhiko   +34 more
openaire   +1 more source

Cardiolipin deficiency disrupts electron transport chain and drives steatohepatitis. [PDF]

Elife
Brothwell MJ, Cao G, Maschek JA, Poss AM, Peterlin AD, Wang L, Baker TB, Shahtout JL, Siripoksup P, Pearce QJ, Johnson JM, Finger FM, Prola A, Pellizzari SA, Hale GL, Manuel AM, Watanabe S, Miranda ER, Affolter KE, Tippetts TS, Nikolova LS, Choi RH, Decker ST, Patil M, Catrow JL, Holland WL, Nowinski SM, Lark DS, Fisher-Wellman KH, Mimche PN, Evason K, Cox JE, Summers SA, Gerhart-Hines Z, Funai K.   +34 more
europepmc   +1 more source

Preclinical and first-in-human evidence of 4-hydroxybenzoic acid for mitochondrial COQ2 deficiency. [PDF]

Brain
Distelmaier F, Corral-Sarasa J, Jiménez-Sánchez L, Díaz-Casado ME, Rohmann M, Seibt A, Herebian D, Smits SHJ, Münch J, Mayatepek E, Breitkreutz J, Husain RA, López-Herrador S, González-García P, López LC.   +14 more
europepmc   +1 more source

Hydroxyhydroquinone as a novel modulator of mitochondrial metabolism with potential to restore CoQ10 homeostasis. [PDF]

Mol Cell Biochem
Dos Santos PR, Rastegar J, Roesch-Ely M.
europepmc   +1 more source

Mitochondrial NAD kinase Pos5 is required for CoQ biosynthesis in yeasts. [PDF]

PLoS One
Nishihara S, Nishida I, Matsuo Y, Kaino T, Kawamukai M.   +4 more
europepmc   +1 more source

Fmp30p is a mitochondrial phosphatidylinositol hydrolase that modulates CoQ biosynthesis. [PDF]

Nat Commun
Baker ZN, Guerra RM, Rogers SW, Pagliarini DJ.   +3 more
europepmc   +1 more source