NP_001164062.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
28,568 Da
NCBI Official Full Name
NFU1 iron-sulfur cluster scaffold homolog, mitochondrial isoform 1
NCBI Official Synonym Full Names
NFU1 iron-sulfur cluster scaffold homolog (S. cerevisiae)
NCBI Protein Information
NFU1 iron-sulfur cluster scaffold homolog, mitochondrial; mHIRIP5; HIRA-interacting protein 5; histone cell cycle regulation defective interacting protein 5
UniProt Protein Name
NFU1 iron-sulfur cluster scaffold homolog, mitochondrial
UniProt Synonym Protein Names
HIRA-interacting protein 5; mHIRIP5
UniProt Synonym Gene Names
UniProt Entry Name
NFU1_MOUSE
UniProt Comments for NFU1
NFU1: Iron-sulfur cluster scaffold protein which can assemble [4Fe-2S] clusters and deliver them to target proteins. Defects in NFU1 are the cause of multiple mitochondrial dysfunctions syndrome type 1 (MMDS1). A severe disorder of systemic energy metabolism, resulting in weakness, respiratory failure, lack of neurologic development, lactic acidosis, hyperglycinemia and early death. Some patients show failure to thrive, pulmonary hypertension, hypotonia and irritability. Biochemical features include severe combined deficiency of the 2-oxoacid dehydrogenases, defective lipoic acid synthesis and reduction in activity of mitochondrial respiratory chain complexes. Belongs to the NifU family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: Mitochondrial
Cellular Component: nucleoplasm; mitochondrion; cytoplasm; cytosol; nucleus
Molecular Function: protein binding; iron ion binding; 4 iron, 4 sulfur cluster binding; iron-sulfur cluster binding
Biological Process: iron-sulfur cluster assembly
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Diseases associated with NFU1 elisa kit
Organs/Tissues associated with NFU1 elisa kit
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