NUBPL: a mitochondrial Complex I deficiency disorder

Các tác giả

  • Muna Abed Rabbo University of British Columbia, Canada
  • Johnny Stiban Birzeit University, Palestine

DOI:

https://doi.org/10.26124/bec:2022-0003

Từ khóa:

Complex I, NUBPL, IND1, iron-sulfur clusters, mtDNA helicase, bioenergetics

Tóm tắt

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.

Cite:

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

Lượt tải xuống

Tệp bổ sung

Đã Xuất bản

2022-06-28

Số

Tập 2022

Chuyên mục

Living Communications

Danh mục

Giấy phép

Bản quyền (c) {copyrightHolder}

Creative Commons License

Tác phẩm này được cấp phép theo Giấy phép Creative Commons Ghi công-Phi thương mại-Không phái sinh 4.0 Quốc tế.

Cách trích dẫn

Abed Rabbo, M., & Stiban, J. (2022). NUBPL: a mitochondrial Complex I deficiency disorder. Bioenergetics Communications, 2022, 3. https://doi.org/10.26124/bec:2022-0003