Proteomics

Dataset Information

0

Human Mitochondria NFS1-ISCU-FXN XL-MS


ABSTRACT: Frataxin (FXN) is involved in mitochondrial iron-sulfur (Fe-S) cluster biogenesis and serves to accelerate Fe-S cluster formation. FXN deficiency is associated with Friedreich ataxia, a neurodegenerative disease. Using chemical cross-linking (XL) accompanied with LC-MS/MS, we studied the the interaction between the core complexes Acp-ISD11-NFS1 (AIN) and AIN-ISCU (AINU) with FXN.

INSTRUMENT(S): LTQ Orbitrap

ORGANISM(S): Homo Sapiens (human)

DISEASE(S): Friedreich Ataxia

SUBMITTER: Kai Cai  

LAB HEAD: John L. Markley

PROVIDER: PXD006938 | Pride | 2018-07-11

REPOSITORIES: Pride

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Publications

Architectural Features of Human Mitochondrial Cysteine Desulfurase Complexes from Crosslinking Mass Spectrometry and Small-Angle X-Ray Scattering.

Cai Kai K   Frederick Ronnie O RO   Dashti Hesam H   Markley John L JL  

Structure (London, England : 1993) 20180705 8


Cysteine desulfurase plays a central role in mitochondrial iron-sulfur cluster biogenesis by generating sulfur through the conversion of L-cysteine to L-alanine and by serving as the platform for assembling other components of the biosynthetic machinery, including ISCU, frataxin, and ferredoxin. The human mitochondrial cysteine desulfurase complex consists of two copies each of NFS1, ISD11, and acyl carrier protein. We describe results from chemical crosslinking coupled with tandem mass spectrom  ...[more]

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