Property Summary

NCBI Gene PubMed Count 24
Grant Count 16
R01 Count 12
Funding $802,815.1
PubMed Score 37.54
PubTator Score 66.53

Knowledge Summary

Patent

No data available

Expression

  Differential Expression (4)

Disease log2 FC p
osteosarcoma 1.349 0.000
medulloblastoma -1.200 0.000
medulloblastoma, large-cell -1.400 0.000
ovarian cancer 1.200 0.000

Gene RIF (10)

PMID Text
26342079 Our findings highlight that the ISD11 R68A/R68L mutation display reduced affinity to form a stable subcomplex with NFS1, and thereby fails to prevent NFS1 aggregation resulting in impairment of the Fe-S cluster biogenesis
24217246 The data presented here show that the Isu1 suppressor mimics the frataxin effects on Nfs1, explaining the bypassing activity.
23940031 NFS1 binds preferentially to the D-state of ISCU while mtHSP70 binds preferentially to the D-state of ISCU and HSC20 binds preferentially to the S-state of ISCU.
23593335 the interaction of NFS1 and MOCS3 in the cytosol of human cells, is reported.
22689995 Nfs1, the cysteine desulfurase responsible for providing sulfur for cluster formation, is required for the increased Isu stability occurring after disruption of cluster formation on or transfer from Isu
20877624 Observational study of gene-disease association. (HuGE Navigator)
19720832 Icp55 protease and its substrate Nfs1 appear to be dual distributed between the nucleus and mitochondria
18650437 Nfs1 acts as a sulfur donor for MOCS3, a protein involved in molybdenum cofactor biosynthesis
16847322 Results show that human Nfs1 is required inside mitochondria for efficient maturation of cellular iron/sulfur proteins.
16527810 the cytosolic form of ISCS is a functional cysteine desulfurase that can collaborate with cytosolic ISCU to promote de novo iron-sulfur cluster formation

AA Sequence

MLLRAAWRRAAVAVTAAPGPKPAAPTRGLRLRVGDRAPQSAVPADTAAAPEVGPVLRPLYMDVQATTPLD      1 - 70
PRVLDAMLPYLINYYGNPHSRTHAYGWESEAAMERARQQVASLIGADPREIIFTSGATESNNIAIKGVAR     71 - 140
FYRSRKKHLITTQTEHKCVLDSCRSLEAEGFQVTYLPVQKSGIIDLKELEAAIQPDTSLVSVMTVNNEIG    141 - 210
VKQPIAEIGRICSSRKVYFHTDAAQAVGKIPLDVNDMKIDLMSISGHKIYGPKGVGAIYIRRRPRVRVEA    211 - 280
LQSGGGQERGMRSGTVPTPLVVGLGAACEVAQQEMEYDHKRISKLSERLIQNIMKSLPDVVMNGDPKHHY    281 - 350
PGCINLSFAYVEGESLLMALKDVALSSGSACTSASLEPSYVLRAIGTDEDLAHSSIRFGIGRFTTEEEVD    351 - 420
YTVEKCIQHVKRLREMSPLWEMVQDGIDLKSIKWTQH                                     421 - 457
//

Text Mined References (27)

PMID Year Title
26342079 2015 Mapping Key Residues of ISD11 Critical for NFS1-ISD11 Subcomplex Stability: IMPLICATIONS IN THE DEVELOPMENT OF MITOCHONDRIAL DISORDER, COXPD19.
25944712 2015 N-terminome analysis of the human mitochondrial proteome.
24217246 2013 Frataxin directly stimulates mitochondrial cysteine desulfurase by exposing substrate-binding sites, and a mutant Fe-S cluster scaffold protein with frataxin-bypassing ability acts similarly.
23940031 2013 Human mitochondrial chaperone (mtHSP70) and cysteine desulfurase (NFS1) bind preferentially to the disordered conformation, whereas co-chaperone (HSC20) binds to the structured conformation of the iron-sulfur cluster scaffold protein (ISCU).
23593335 2013 The L-cysteine desulfurase NFS1 is localized in the cytosol where it provides the sulfur for molybdenum cofactor biosynthesis in humans.
22689995 2012 Cysteine desulfurase Nfs1 and Pim1 protease control levels of Isu, the Fe-S cluster biogenesis scaffold.
21269460 2011 Initial characterization of the human central proteome.
20877624 2010 Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression.
20873749 2010 Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex.
19720832 2009 Dual targeting of Nfs1 and discovery of its novel processing enzyme, Icp55.
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