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Abed Rabbo 2022 BEC

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Bioenergetics Communications        
Gnaiger 2020 BEC MitoPathways
       
Gnaiger Erich et al ― MitoEAGLE Task Group (2020) Mitochondrial physiology. Bioenerg Commun 2020.1.
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Bioenergetics Communications
Publications in the MiPMap
Abed Rabbo M, Stiban J (2022) NUBPL: a mitochondrial Complex I deficiency disorder. https://doi.org/10.26124/bec:2022-0003

» Bioenerg Commun 2022.2. Open Access pdf published online 2022-06-28 »Watch the presentation«

Abed Rabbo Muna, Stiban Johnny (BEC 2022.03) Bioenerg Commun

Abstract: BEC.png https://doi.org/10.26124/bec:2022-0003 Mitochondrial ailments are diverse and devastating. Defects in mitochondrial DNA or its products lead to a wide range of deficiencies in the mitochondrial electron transfer system and its ensuing energy transformation. Accessory proteins required for the assembly and function of the respiratory complexes are also required for healthy, coupled, and energy-transforming mitochondria. Recently, the protein nucleotide-binding protein-like (NUBPL or IND1) was identified as an iron-sulfur cluster transfer protein specifically for Complex I. Since the presence of multiple iron-sulfur clusters in Complex I is necessary for its activity, deficiency in NUBPL leads to severely dysfunctional mitochondria, with upregulated compensatory Complex II activity. Here we present a short review of the debilitating disease related to NUBPL deficiency.
Keywords: Complex I, NUBPL, IND1, iron-sulfur clusters, mtDNA helicase, bioenergetics
Bioblast editor: Tindle-Solomon Lisa

ORCID: ORCID.png Stiban Johnny

Preprint

» Abed Rabbo 2022 MitoFit


Labels: MiParea: nDNA;cell genetics  Pathology: Inherited 

Organism: Human  Tissue;cell: Heart, Skeletal muscle, Nervous system, Liver 




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