Gene name: NFS1

Uniprot entry:

Q9Y697

Protein names:

Cysteine desulfurase, mitochondrial (EC 2.8.1.7)

Protein sequence:

1_MLLRA 6_ AWRRA 11_ AVAVT 16_ AAPGP 21_ KPAAP 26_ TRGLR 31_ LRVGD 36_ RAPQS 41_ AVPAD 46_ TAAAP 51_ EVGPV 56_ LRPLY 61_ MDVQA 66_ TTPLD 71_ PRVLD 76_ AMLPY 81_ LINYY 86_ GNPHS 91_ RTHAY 96_ GWESE 101_ AAMER 106_ ARQQV 111_ ASLIG 116_ ADPRE 121_ IIFTS 126_ GATES 131_ NNIAI 136_ KGVAR 141_ FYRSR 146_ KKHLI 151_ TTQTE 156_ HKCVL 161_ DSCRS 166_ LEAEG 171_ FQVTY 176_ LPVQK 181_ SGIID 186_ LKELE 191_ AAIQP 196_ DTSLV 201_ SVMTV 206_ NNEIG 211_ VKQPI 216_ AEIGR 221_ ICSSR 226_ KVYFH 231_ TDAAQ 236_ AVGKI 241_ PLDVN 246_ DMKID 251_ LMSIS 256_ GHKIY 261_ GPKGV 266_ GAIYI 271_ RRRPR 276_ VRVEA 281_ LQSGG 286_ GQERG 291_ MRSGT 296_ VPTPL 301_ VVGLG 306_ AACEV 311_ AQQEM 316_ EYDHK 321_ RISKL 326_ SERLI 331_ QNIMK 336_ SLPDV 341_ VMNGD 346_ PKHHY 351_ PGCIN 356_ LSFAY 361_ VEGES 366_ LLMAL 371_ KDVAL 376_ SSGSA 381_ CTSAS 386_ LEPSY 391_ VLRAI 396_ GTDED 401_ LAHSS 406_ IRFGI 411_ GRFTT 416_ EEEVD 421_ YTVEK 426_ CIQHV 431_ KRLRE 436_ MSPLW 441_ EMVQD 446_ GIDLK 451_SIKWT

Protein annotations

Protein functions:

1: Cysteine desulfurase, of the core iron-sulfur cluster (ISC) assembly complex, that catalyzes the desulfuration of L-cysteine to L-alanine, as component of the cysteine desulfurase complex, leading to the formation of a cysteine persulfide intermediate at the active site cysteine residue and participates in the [2Fe-2S] clusters assembly on the scaffolding protein ISCU (PubMed:18650437, PubMed:29097656, PubMed:31101807). The persulfide is then transferred on the flexible Cys loop from the catalytic site of NFS1 to the surface of NFS1 (PubMed:29097656). After the NFS1-linked persulfide sulfur is transferred to one of the conserved Cys residues of the scaffold, a reaction assisted by FXN (By similarity). The core iron-sulfur cluster (ISC) assembly complex is involved in the de novo synthesis of a [2Fe-2S] cluster, the first step of the mitochondrial iron-sulfur protein biogenesis. This process is initiated by the cysteine desulfurase complex (NFS1:LYRM4:NDUFAB1) that produces persulfide which is delivered on the scaffold protein ISCU in a FXN-dependent manner. Then this complex is stabilized by FDX2 which provides reducing equivalents to accomplish the [2Fe-2S] cluster assembly. Finally, the [2Fe-2S] cluster is transferred from ISCU to chaperone proteins, including HSCB, HSPA9 and GLRX5 (By similarity)

2: May catalyze the desulfuration of L-cysteine to L-alanine as component of the cysteine desulfurase complex (NFS1:LYRM4), leading to the formation of a cysteine persulfide intermediate (PubMed:16527810, PubMed:18650437). Acts as a sulfur donor for MOCS3 by transferring the sulfur of the cysteine persulfide intermediate on MOCS3 (PubMed:18650437, PubMed:23593335)