Complex: MITOCHONDRIAL 2FE-2S ASSEMBLY COMPLEX

General Information

Name

MITOCHONDRIAL 2FE-2S ASSEMBLY COMPLEX

Species

D. melanogaster

Symbol

NIAUF

FlyBase ID

FBgg0001989

Date last reviewed

2025-01-31

Number of members

Description

The Mitochondrial 2FE-2S assembly complex is the core complex involved in the synthesis of organometallic Fe-S clusters in the mitochondrial matrix. This complex mediates the formation of 2Fe-2S clusters by the cysteine-derived sulfur and ferrous iron. 2Fe-2S clusters are synthesized on the scaffold protein encoded by IscU. Downstream, Fe-S clusters are incorporated into various proteins and are required for a variety of processes such as electron transfer, catalysis and structural roles. (Adapted from FBrf0238243.)

Notes on Group

In humans, the Mitochondrial 2FE-2S assembly complex consists of ISCU, NFS1, LYRM4 (ISD11), NDUFAB1 (acyl carrier protein, ACP) and FXN (frataxin). Human NDUFAB1/fly ND-ACP is an accessory subunit of Mitochondrial Complex I, but human NDUFAB1 has also been identified as an essential stabilizing component of the Mitochondrial 2FE-2S iron-sulfur assembly complex, with the 4'-phosphopantetheine-conjugated acyl-group of NDUFAB1 occupying the hydrophobic core of LYRM4 (PMID:28634302). ND-ACP has not been characterised as a member of this complex in flies, but FBrf0216491 shows an interaction between ND-ACP (ortholog of NDUFAB1) bcn92 (ortholog of LYRM4). This has been used as evidence to include it in the MITOCHONDRIAL 2FE-2S ASSEMBLY COMPLEX gene group.

Source Material

The MITOCHONDRIAL 2FE-2S ASSEMBLY COMPLEX Gene Group has been compiled using the following publication(s): Marelja et al., 2018 and Guruharsha et al., 2011 .

Key Gene Ontology (GO) terms

Molecular Function

iron-sulfur cluster chaperone activity

Biological Process

[2Fe-2S] cluster assembly

Cellular Component

mitochondrial [2Fe-2S] assembly complex

mitochondrial matrix

Related Gene Groups

Members (5)

For all members: