Svetlana Konovalova
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Explore the profile of Svetlana Konovalova including associated specialties, affiliations and a list of published articles.
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20
Citations
322
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Recent Articles
1.
Manjunath P, Stojkovic G, Euro L, Konovalova S, Wanrooij S, Koski K, et al.
Protein Sci
. 2024 Oct;
33(11):e5190.
PMID: 39445986
Human nucleotide exchange factors GRPEL1 and GRPEL2 play pivotal roles in the ADP-ATP exchange within the protein folding cycle of mitochondrial HSP70 (mtHSP70), a crucial chaperone facilitating protein import into...
2.
Somborac T, Lutfullahoglu Bal G, Fatima K, Vihinen H, Paatero A, Jokitalo E, et al.
PLoS One
. 2023 Dec;
18(12):e0295047.
PMID: 38039321
Peroxisomes are membrane-enclosed organelles with important roles in fatty acid breakdown, bile acid synthesis and biosynthesis of sterols and ether lipids. Defects in peroxisomes result in severe genetic diseases, such...
3.
Konovalova S, Torregrosa-Munumer R, Manjunath P, Liu X, Baral S, Fatima K, et al.
Proc Natl Acad Sci U S A
. 2023 Jul;
120(30):e2210599120.
PMID: 37463214
Cardiolipin (CL) is an essential phospholipid for mitochondrial structure and function. Here, we present a small mitochondrial protein, NERCLIN, as a negative regulator of CL homeostasis and mitochondrial ultrastructure. Primate-specific...
4.
Dohla J, Kuuluvainen E, Gebert N, Amaral A, Englund J, Gopalakrishnan S, et al.
Nat Cell Biol
. 2022 Feb;
24(2):148-154.
PMID: 35165416
Metabolic characteristics of adult stem cells are distinct from their differentiated progeny, and cellular metabolism is emerging as a potential driver of cell fate conversions. How these metabolic features are...
5.
Harjuhaahto S, Rasila T, Molchanova S, Woldegebriel R, Kvist J, Konovalova S, et al.
Neurobiol Dis
. 2020 May;
141:104940.
PMID: 32437855
Mitochondrial intermembrane space proteins CHCHD2 and CHCHD10 have roles in motor neuron diseases such as amyotrophic lateral sclerosis, spinal muscular atrophy and axonal neuropathy and in Parkinson's disease. They form...
6.
Nevanlinna V, Konovalova S, Ceulemans B, Muona M, Laari A, Hilander T, et al.
Eur J Med Genet
. 2019 Sep;
63(3):103766.
PMID: 31536827
Pontocerebellar hypoplasia type 6 (PCH6) is a rare infantile-onset progressive encephalopathy caused by biallelic mutations in RARS2 that encodes the mitochondrial arginine-tRNA synthetase enzyme (mtArgRS). The clinical presentation overlaps that...
7.
Konovalova S
J Vis Exp
. 2019 Mar;
(144).
PMID: 30829336
Mitochondrial respiration is performed by oxidative phosphorylation (OXPHOS) complexes within mitochondria. Internal and environmental factors can perturb the assembly and stability of OXPHOS complexes. This protocol describes the analysis of...
8.
Sommerville E, Zhou X, Olahova M, Jenkins J, Euro L, Konovalova S, et al.
Hum Mol Genet
. 2018 Oct;
28(2):258-268.
PMID: 30285085
Recessively inherited variants in AARS2 (NM_020745.2) encoding mitochondrial alanyl-tRNA synthetase (mt-AlaRS) were first described in patients presenting with fatal infantile cardiomyopathy and multiple oxidative phosphorylation defects. To date, all described...
9.
Konovalova S, Liu X, Manjunath P, Baral S, Neupane N, Hilander T, et al.
Redox Biol
. 2018 Aug;
19:37-45.
PMID: 30098457
Mitochondria are central organelles to cellular metabolism. Their function relies largely on nuclear-encoded proteins that must be imported from the cytosol, and thus the protein import pathways are important for...
10.
Hilander T, Konovalova S, Terzioglu M, Tyynismaa H
Curr Protoc Toxicol
. 2018 Aug;
77(1):e56.
PMID: 30063298
Mitochondria are multifunctional organelles with their own genome and protein synthesis machinery. The 13 proteins encoded by mitochondrial DNA (mtDNA) are core subunits of the oxidative phosphorylation (OXPHOS) system producing...