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NFS1

Iron-sulfur clusters are required for the function of many cellular enzymes. The proteins encoded by this gene supply inorganic sulfur to these clusters by removing the sulfur from cysteine, creating alanine in the process. This gene uses alternate in-frame translation initiation sites to generate mitochondrial forms and cytoplasmic/nuclear forms. Selection of the alternative initiation sites is determined by the cytosolic pH. The encoded proteins belong to the class-V family of pyridoxal phosphate-dependent aminotransferases. Alternatively spliced transcript variants have been described.

Full Name

NFS1, Cysteine Desulfurase

Function

Catalyzes the removal of elemental sulfur from cysteine to produce alanine (PubMed:18650437).
It supplies the inorganic sulfur for iron-sulfur (Fe-S) clusters. May be involved in the biosynthesis of molybdenum cofactor (PubMed:18650437).

Biological Process

[2Fe-2S] cluster assemblyIEA:InterPro
Iron incorporation into metallo-sulfur clusterManual Assertion Based On ExperimentIDA:HGNC-UCL
Iron-sulfur cluster assemblyManual Assertion Based On ExperimentIBA:GO_Central
Mo-molybdopterin cofactor biosynthetic processIEA:UniProtKB-KW

Cellular Location

Isoform Mitochondrial
Mitochondrion
Isoform Cytoplasmic
Cytoplasm
Nucleus

Involvement in disease

Combined oxidative phosphorylation deficiency 52 (COXPD52):
An autosomal recessive mitochondrial disorder with onset in infancy, characterized by lactic acidemia, hypotonia, respiratory chain complex II and III deficiency, multisystem organ failure and abnormal mitochondria.

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Anti-NFS1 antibodies

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Mouse Anti-NFS1 Recombinant Antibody (CBWJN-1386) (CBMAB-N2324-WJ)

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Target: NFS1

Host: Mouse

Antibody Isotype: IgG1

Specificity: Human, Mouse, Rat

Clone: CBWJN-1386

Application*: WB

Mouse Anti-NFS1 Recombinant Antibody (5D1) (CBMAB-N2323-WJ)

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Target: NFS1

Host: Mouse

Antibody Isotype: IgG2a

Specificity: Human

Clone: 5D1

Application*: WB, IH

Mouse Anti-NFS1 Recombinant Antibody (2H5) (CBMAB-N2322-WJ)

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Target: NFS1

Host: Mouse

Antibody Isotype: IgG2b

Specificity: Human

Clone: 2H5

Application*: WB, IH

Mouse Anti-NFS1 Recombinant Antibody (1D5) (CBMAB-N2321-WJ)

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Target: NFS1

Host: Mouse

Specificity: Human

Clone: 1D5

Application*: E, WB

Mouse Anti-NFS1 Recombinant Antibody (CBWJN-0317) (CBMAB-N0338-WJ)

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Target: NFS1

Host: Mouse

Antibody Isotype: IgG1

Specificity: Human

Clone: CBWJN-0317

Application*: DB, WB

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(P): Predicted

* Abbreviations

AActivation
AGAgonist
APApoptosis
BBlocking
BABioassay
BIBioimaging
CImmunohistochemistry-Frozen Sections
CIChromatin Immunoprecipitation
CTCytotoxicity
CSCostimulation
DDepletion
DBDot Blot

EELISA
ECELISA(Cap)
EDELISA(Det)
ESELISpot
EMElectron Microscopy
FFlow Cytometry
FNFunction Assay
GSGel Supershift
IInhibition
IAEnzyme Immunoassay
ICImmunocytochemistry
IDImmunodiffusion
IEImmunoelectrophoresis

IFImmunofluorescence
IGImmunochromatography
IHImmunohistochemistry
IMImmunomicroscopy
IOImmunoassay
IPImmunoprecipitation
ISIntracellular Staining for Flow Cytometry
LALuminex Assay
LFLateral Flow Immunoassay
MMicroarray
MCMass Cytometry/CyTOF
MDMeDIP

MSElectrophoretic Mobility Shift Assay
NNeutralization
PImmunohistologyp-Paraffin Sections
PAPeptide Array
PEPeptide ELISA
PLProximity Ligation Assay
RRadioimmunoassay
SStimulation
SESandwich ELISA
SHIn situ hybridization
TCTissue Culture
WBWestern Blot

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