Abed Rabbo 2022 BEC

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Bioenergetics Communications        
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Gnaiger Erich et al ― MitoEAGLE Task Group (2020) Mitochondrial physiology. Bioenerg Commun 2020.1.
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Abed Rabbo M, Stiban J (2022) NUBPL: a mitochondrial Complex I deficiency disorder. Bioenerg Commun 2022.3. https://doi.org/10.26124/bec:2022-0003

Β» Bioenerg Commun 2022.3. Open Access pdf published online 2022-06-28 Β»Watch the presentationΒ«

Abed Rabbo Muna, Stiban Johnny (2022) 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.
β€’ Keywords: Complex I, NUBPL, IND1, iron-sulfur clusters, mtDNA helicase, bioenergetics
β€’ Bioblast editor: Tindle-Solomon Lisa

ORCID: ORCID.png Stiban Johnny


Β» 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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