NFU1 : NFU1 iron-sulfur cluster scaffold

Description

The NFU1 gene encodes a crucial protein involved in the assembly of iron-sulfur clusters (ISCs), essential cofactors for a vast array of cellular processes. Located in the mitochondria, NFU1 participates in the maturation of ISC-containing proteins, playing a vital role in cellular respiration, DNA replication, and nucleotide metabolism. Mutations in NFU1 can lead to various mitochondrial disorders, highlighting its importance in maintaining cellular function.

Associated Diseases

• Friedreich‘s Ataxia
• Mitochondrial Encephalomyopathy with Lactic Acidosis and Stroke-like Episodes (MELAS)
• Leigh Syndrome
• Multiple Mitochondrial Dysfunction Syndromes

Did you know?

NFU1 is a highly conserved gene across species, suggesting its fundamental importance in cellular function.