Cysteine desulfurase, mitochondrial (NFS1, NIFS, HUSSY-08)

Function

• catalyzes removal of elemental sulfur from cysteine to produce alanine
• supplies inorganic sulfur for iron-sulfur (Fe-S) clusters
• may be involved in the biosynthesis of molybdenum cofactor
• binds ISCU/NIFUN
• forms a complex with LYRM4

L-cysteine + [enzyme]-cysteine = L-alanine + [enzyme]-S-sulfanylcysteine

Cofactor: pyridoxal phosphate (putative)

Kinetic parameters:

• KM=434.75 uM for L-cysteine (as complex with LYRM4)

Structure

• belongs to the class-V pyridoxal-phosphate-dependent aminotransferase family, NifS/IscS subfamily

Compartment

• mitochondria, cytoplasm, nucleus Event=Alternative initiation;
• named isoforms=2
• mitochondrial, cytoplasmic

Expression

• predominantly expressed in heart & skeletal muscle
• also found in brain, liver & pancreas

More general terms

• mitochondrial protein
• sulfurtransferase

Additional terms

• HUSSY protein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=9054
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:9054
• OMIM: https://mirror.omim.org/entry/603485
• UniProt: http://www.uniprot.org/uniprot/Q9Y697.html