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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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NUBP1
NUBP iron-sulfur cluster assembly factor 1, cytosolic
Chromosome 16 · 16p13.13
NCBI Gene: 4682Ensembl: ENSG00000103274.12HGNC: HGNC:8041UniProt: P53384
43PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
regulation of cell growthprotein bindingcytosoliron-sulfur cluster bindingimmune system diseaseneurodegenerative diseasesyndactyly type 1Syndactyly type 2
✦AI Summary

NUBP1 (nucleotide-binding protein 1) is a cytosolic P-loop NTPase that functions as a central component of the cytosolic iron-sulfur (Fe/S) cluster assembly (CIA) machinery 1. As part of the NUBP1-NUBP2 heterotetramer, it forms a Fe/S scaffold complex essential for de novo assembly of [4Fe-4S] clusters and their transfer to target apoproteins 1. NUBP1 receives [2Fe-2S] clusters from glutaredoxin-3 (GLRX3), which acts as a cluster chaperone 2. The transfer mechanism involves cooperative cluster donation from GLRX3 and electron provision from anamorsin to reductively couple two [2Fe-2S] clusters into [4Fe-4S] on NUBP1's N-terminal and C-terminal binding motifs 3. Beyond Fe/S cluster maturation, NUBP1 regulates centrosome dynamics and microtubule organization, with critical roles in cell polarity maintenance 4. Mutations in NUBP1 cause lung hypoplasia with increased apoptosis in distal progenitor cells and disrupted localization of polarity proteins, demonstrating its importance in developmental morphogenesis 4. NUBP1 is part of a conserved eukaryotic gene family sharing ATP/GTP-binding motifs and NUBP/MRP signature sequences 5.

Sources cited
1
NUBP1 is a component of CIA machinery required for extramitochondrial Fe/S protein maturation and forms heterotetramer with NUBP2 for cluster assembly and transfer
PMID: 18573874
2
GLRX3 transfers [2Fe-2S] clusters to NUBP1 to form [4Fe-4S] clusters in cytosolic iron-sulfur assembly
PMID: 32429669
3
Hetero-tetrameric complex of anamorsin and GLRX3 orchestrates [4Fe-4S] cluster assembly on NUBP1's N-terminal binding site
PMID: 36916754
4
GLRX3 domains cooperatively transfer [2Fe-2S] clusters to NUBP1 for [4Fe-4S] cluster formation through domain-specific mechanisms
PMID: 41092652
5
NUBP1 mutation causes lung hypoplasia, increased apoptosis in distal progenitors, and disrupts centrosome dynamics and cell polarity protein localization
PMID: 23028652
6
NUBP1 is a P-loop nucleotide-binding protein with conserved ATP/GTP-binding motifs and unique N-terminal cysteines, mapped to chromosome 16
PMID: 10486206
Disease Associationsⓘ20
immune system diseaseOpen Targets
0.13Weak
neurodegenerative diseaseOpen Targets
0.08Suggestive
syndactyly type 1Open Targets
0.07Suggestive
Syndactyly type 2Open Targets
0.07Suggestive
syndactyly type 4Open Targets
0.07Suggestive
colorectal carcinomaOpen Targets
0.07Suggestive
polydactyly of a triphalangeal thumbOpen Targets
0.07Suggestive
syndactyly type 3Open Targets
0.07Suggestive
Split hand-split foot malformationOpen Targets
0.07Suggestive
syndactyly type 5Open Targets
0.07Suggestive
mesoaxial synostotic syndactyly with phalangeal reductionOpen Targets
0.07Suggestive
PolysyndactylyOpen Targets
0.06Suggestive
polysyndactyly 4Open Targets
0.06Suggestive
brachydactyly-syndactyly syndromeOpen Targets
0.06Suggestive
brachydactyly type A2Open Targets
0.06Suggestive
synpolydactyly type 1Open Targets
0.06Suggestive
Triphalangeal thumb - polysyndactyly syndromeOpen Targets
0.06Suggestive
brachydactyly type A7Open Targets
0.06Suggestive
triphalangeal thumb-polysyndactyly syndromeOpen Targets
0.06Suggestive
split-foot malformation-mesoaxial polydactyly syndromeOpen Targets
0.06Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CIAO1Protein interaction100%MMS19Protein interaction100%CIAO2BProtein interaction99%CIAO2AProtein interaction99%CIAO3Protein interaction96%CIAPIN1Protein interaction90%
Tissue Expression6 tissues
Heart
100%
Liver
61%
Lung
58%
Ovary
58%
Bone Marrow
56%
Brain
47%
Gene Interaction Network
Click a node to explore
NUBP1CIAO1MMS19CIAO2BCIAO2ACIAO3CIAPIN1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt P53384
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.50LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.17 [0.92–1.50]
RankingsWhere NUBP1 stands among ~20K protein-coding genes
  • #9,765of 20,598
    Most Researched43
  • #15,160of 17,882
    Most Constrained (LOEUF)1.50
Genes detectedNUBP1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Human glutaredoxin 3: multiple domains for a unique function.
PMID: 41092652
J Inorg Biochem · 2025
1.00
2
Two novel mouse genes--Nubp2, mapped to the t-complex on chromosome 17, and Nubp1, mapped to chromosome 16--establish a new gene family of nucleotide-binding proteins in eukaryotes.
PMID: 10486206
Genomics · 1999
0.90
3
Nubp1 is required for lung branching morphogenesis and distal progenitor cell survival in mice.
PMID: 23028652
PLoS One · 2012
0.80
4
GLRX3 Acts as a [2Fe-2S] Cluster Chaperone in the Cytosolic Iron-Sulfur Assembly Machinery Transferring [2Fe-2S] Clusters to NUBP1.
PMID: 32429669
J Am Chem Soc · 2020
0.70
5
Unraveling the mechanism of [4Fe-4S] cluster assembly on the N-terminal cluster binding site of NUBP1.
PMID: 36916754
Protein Sci · 2023
0.60