The NFU1 gene provides instructions for making a protein involved in the formation of molecules called iron-sulfur (Fe-S) clusters. These clusters are attached to certain other proteins and are required for their proper function.
Two versions (isoforms) of the NFU-1 protein are produced from the NFU1 gene. One version is found in cellular structures called mitochondria. Mitochondria are the energy-producing centers of cells. In these structures, several proteins carry out a series of chemical steps to convert the energy in food into a form that cells can use. Many of the proteins involved in this process require Fe-S clusters to function, including protein complexes called complex I, complex II, and complex III.
Fe-S clusters are also required for another mitochondrial protein to function; this protein is involved in the modification of additional proteins that aid in energy production in mitochondria, including the pyruvate dehydrogenase complex and the alpha-ketoglutarate dehydrogenase complex. This modification is also critical to the function of the glycine cleavage system, a set of proteins that breaks down a protein building block (amino acid) called glycine when levels become too high.
The other version of the NFU-1 protein is found in the fluid-filled space inside the cell (the cytoplasm). While this protein is likely involved in Fe-S cluster formation in the cytoplasm, the role of this isoform is not well understood.
Other Names
CGI-33; HIRA-interacting protein 5; HIRIP; HIRIP5; iron-sulfur cluster scaffold protein; MMDS1; Nfu; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial isoform 1; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial isoform 2; NFU1 iron-sulfur cluster scaffold homolog, mitochondrial isoform 3; NFU1_HUMAN; NifU; NifU-like C-terminal domain containing; NIFUC
Related Genetics Topics
Identifiers
- NCBI Gene: 27247
- OMIM: 608100
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
