Stiban 2022 Abstract Bioblast

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7.5. «10+5»
Johnny Stiban
Abed Rabbo M, Stiban Johnny (2022) NUBPL: a mitochondrial Complex I deficiency disorder. Bioblast 2022: BEC Inaugural Conference. In: https://doi.org/10.26124/bec:2022-0001
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Link: Bioblast 2022: BEC Inaugural Conference

Abed Rabbo Muna, Stiban Johnny (2022)

Event: Bioblast 2022

Mitochondrial ailments are diverse and devastating. Defects in mitochondrial DNA or its products lead to wide range of deficiencies in the mitochondrial electron transfer system and its ensuing energy production. Accessory proteins required for the assembly and function of the respiratory complexes are also required for healthy, coupled, and energy-producing 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


Affiliations

Abed Rabbo M1, Stiban J2Superscript text
  1. Department of Medical Genetics, University of British Columbia, Canada
  2. Department of Biology and Biochemistry, Birzeit University, Palestine - jstiban@birzeit.edu

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