KIFBP Gene

Name kinesin family binding protein
Description This gene encodes a kinesin family member 1 binding protein that is characterized by two tetratrico peptide repeats. The encoded protein localizes to the mitochondria and may be involved in regulating transport of the mitochondria. Mutations in this gene are associated with Goldberg-Shprintzen megacolon syndrome. [provided by RefSeq, Mar 2010]
Summary
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By binding directly to the motor domains of specific kinesins, KBP prevents their association with microtubules, thereby modulating the activity of motors involved in vesicular transport and in microtubule dynamics in neuronal cells."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "1"}]}, {"type": "t", "text": " In developing neurons, this inhibitory role is crucial for controlling synaptic vesicle transport and ensuring proper axonal growth, while in embryonic stem cells, regulated degradation of KBP is essential to prevent unscheduled increases in mitochondrial biogenesis and oxidative stress."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "3"}]}, {"type": "t", "text": " During neural development, loss‐of‐function mutations in KIAA1279 cause Goldberg–Shprintzen syndrome—a disorder marked by intellectual disability, microcephaly, Hirschsprung disease, and other neuronal abnormalities—underscoring KBP’s vital role in both central and peripheral nervous system development."}, {"type": "fg", "children": [{"type": "fg_fs", "start_ref": "4", "end_ref": "6"}]}, {"type": "t", "text": ""}]}, {"type": "t", "text": "\n \n "}, {"type": "p", "children": [{"type": "t", "text": "\n In addition, KBP functions as a “protein buffer” during mitosis by reducing the activities of kinesins such as KIF18A and KIF15, thereby preventing excessive microtubule binding that would otherwise disrupt proper chromosome alignment and spindle dynamics."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "7"}]}, {"type": "t", "text": " KBP also interacts with other kinesin family members, including KIF1Bα and the newly identified KLP6, to regulate mitochondrial morphology and transport in neuronal and other cell types."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "2"}, {"type": "fg_f", "ref": "9"}]}, {"type": "t", "text": " Moreover, its interaction with kinesin partners has been implicated in specialized processes such as spermatid elongation during spermatogenesis"}, {"type": "fg", "children": [{"type": "fg_f", "ref": "10"}]}, {"type": "t", "text": "and may also have prognostic or driver roles in malignancies like neuroblastoma."}, {"type": "fg", "children": [{"type": "fg_f", "ref": "11"}]}, {"type": "t", "text": "\n "}]}, {"type": "rg", "children": [{"type": "r", "ref": 1, "children": [{"type": "t", "text": "Josta T Kevenaar, Sarah Bianchi, Myrrhe van Spronsen, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Kinesin-Binding Protein Controls Microtubule Dynamics and Cargo Trafficking by Regulating Kinesin Motor Activity."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Curr Biol (2016)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1016/j.cub.2016.01.048"}], "href": "https://doi.org/10.1016/j.cub.2016.01.048"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "26948876"}], "href": "https://pubmed.ncbi.nlm.nih.gov/26948876"}]}, {"type": "r", "ref": 2, "children": [{"type": "t", "text": "Marcin J Wozniak, Martina Melzer, Cornelia Dorner, et al. "}, {"type": "b", "children": [{"type": "t", "text": "The novel protein KBP regulates mitochondria localization by interaction with a kinesin-like protein."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "BMC Cell Biol (2005)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1186/1471-2121-6-35"}], "href": "https://doi.org/10.1186/1471-2121-6-35"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "16225668"}], "href": "https://pubmed.ncbi.nlm.nih.gov/16225668"}]}, {"type": "r", "ref": 3, "children": [{"type": "t", "text": "Valerio Donato, Massimo Bonora, Daniele Simoneschi, et al. "}, {"type": "b", "children": [{"type": "t", "text": "The TDH-GCN5L1-Fbxo15-KBP axis limits mitochondrial biogenesis in mouse embryonic stem cells."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Nat Cell Biol (2017)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1038/ncb3491"}], "href": "https://doi.org/10.1038/ncb3491"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "28319092"}], "href": "https://pubmed.ncbi.nlm.nih.gov/28319092"}]}, {"type": "r", "ref": 4, "children": [{"type": "t", "text": "Loïc Drévillon, André Megarbane, Bénédicte Demeer, et al. "}, {"type": "b", "children": [{"type": "t", "text": "KBP-cytoskeleton interactions underlie developmental anomalies in Goldberg-Shprintzen syndrome."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Hum Mol Genet (2013)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1093/hmg/ddt083"}], "href": "https://doi.org/10.1093/hmg/ddt083"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "23427148"}], "href": "https://pubmed.ncbi.nlm.nih.gov/23427148"}]}, {"type": "r", "ref": 5, "children": [{"type": "t", "text": "Alice S Brooks, Aida M Bertoli-Avella, Grzegorz M Burzynski, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Homozygous nonsense mutations in KIAA1279 are associated with malformations of the central and enteric nervous systems."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Am J Hum Genet (2005)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1086/431244"}], "href": "https://doi.org/10.1086/431244"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "15883926"}], "href": "https://pubmed.ncbi.nlm.nih.gov/15883926"}]}, {"type": "r", "ref": 6, "children": [{"type": "t", "text": "Hormos Salimi Dafsari, Susan Byrne, Jean-Pierre Lin, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Goldberg-Shprintzen megacolon syndrome with associated sensory motor axonal neuropathy."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Am J Med Genet A (2015)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1002/ajmg.a.36873"}], "href": "https://doi.org/10.1002/ajmg.a.36873"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "25846562"}], "href": "https://pubmed.ncbi.nlm.nih.gov/25846562"}]}, {"type": "r", "ref": 7, "children": [{"type": "t", "text": "Heidi L H Malaby, Megan E Dumas, Ryoma Ohi, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Kinesin-binding protein ensures accurate chromosome segregation by buffering KIF18A and KIF15."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Cell Biol (2019)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1083/jcb.201806195"}], "href": "https://doi.org/10.1083/jcb.201806195"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "30709852"}], "href": "https://pubmed.ncbi.nlm.nih.gov/30709852"}]}, {"type": "r", "ref": 8, "children": [{"type": "t", "text": "Nathalie Brouwers, Nuria Mallol Martinez, Isabelle Vernos "}, {"type": "b", "children": [{"type": "t", "text": "Role of Kif15 and its novel mitotic partner KBP in K-fiber dynamics and chromosome alignment."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "PLoS One (2017)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1371/journal.pone.0174819"}], "href": "https://doi.org/10.1371/journal.pone.0174819"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "28445502"}], "href": "https://pubmed.ncbi.nlm.nih.gov/28445502"}]}, {"type": "r", "ref": 9, "children": [{"type": "t", "text": "Kousuke Tanaka, Yoshimi Sugiura, Ryohei Ichishita, et al. "}, {"type": "b", "children": [{"type": "t", "text": "KLP6: a newly identified kinesin that regulates the morphology and transport of mitochondria in neuronal cells."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "J Cell Sci (2011)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1242/jcs.086470"}], "href": "https://doi.org/10.1242/jcs.086470"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "21693574"}], "href": "https://pubmed.ncbi.nlm.nih.gov/21693574"}]}, {"type": "r", "ref": 10, "children": [{"type": "t", "text": "Mari S Lehti, Noora Kotaja, Anu Sironen "}, {"type": "b", "children": [{"type": "t", "text": "KIF1-binding protein interacts with KIF3A in haploid male germ cells."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Reproduction (2015)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1530/REP-15-0173"}], "href": "https://doi.org/10.1530/REP-15-0173"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "26245936"}], "href": "https://pubmed.ncbi.nlm.nih.gov/26245936"}]}, {"type": "r", "ref": 11, "children": [{"type": "t", "text": "Chen Suo, Wenjiang Deng, Trung Nghia Vu, et al. "}, {"type": "b", "children": [{"type": "t", "text": "Accumulation of potential driver genes with genomic alterations predicts survival of high-risk neuroblastoma patients."}]}, {"type": "t", "text": " "}, {"type": "i", "children": [{"type": "t", "text": "Biol Direct (2018)"}]}, {"type": "t", "text": " DOI: "}, {"type": "a", "children": [{"type": "t", "text": "10.1186/s13062-018-0218-5"}], "href": "https://doi.org/10.1186/s13062-018-0218-5"}, {"type": "t", "text": " PMID: "}, {"type": "a", "children": [{"type": "t", "text": "30012197"}], "href": "https://pubmed.ncbi.nlm.nih.gov/30012197"}]}]}]}
NCBI Gene ID 26128
API
Download Associations
Predicted Functions View KIFBP's ARCHS4 Predicted Functions.
Co-expressed Genes View KIFBP's ARCHS4 Predicted Functions.
Expression in Tissues and Cell Lines View KIFBP's ARCHS4 Predicted Functions.

Functional Associations

KIFBP has 2,364 functional associations with biological entities spanning 6 categories (chemical, disease, phenotype or trait, functional term, phrase or reference, cell line, cell type or tissue, gene, protein or microRNA, sequence feature) extracted from 36 datasets.

Click the + buttons to view associations for KIFBP from the datasets below.

If available, associations are ranked by standardized value

Dataset Summary
Allen Brain Atlas Aging Dementia and Traumatic Brain Injury Tissue Sample Gene Expression Profiles tissue samples with high or low expression of KIFBP gene relative to other tissue samples from the Allen Brain Atlas Aging Dementia and Traumatic Brain Injury Tissue Sample Gene Expression Profiles dataset.
Carcinogenome Chemical Perturbation Carcinogenicity Signatures small molecule perturbations changing expression of KIFBP gene from the Carcinogenome Chemical Perturbation Carcinogenicity Signatures dataset.
CCLE Cell Line Proteomics Cell lines associated with KIFBP protein from the CCLE Cell Line Proteomics dataset.
ChEA Transcription Factor Targets 2022 transcription factors binding the promoter of KIFBP gene in low- or high-throughput transcription factor functional studies from the CHEA Transcription Factor Targets 2022 dataset.
ClinVar Gene-Phenotype Associations 2025 phenotypes associated with KIFBP gene from the curated ClinVar Gene-Phenotype Associations 2025 dataset.
COMPARTMENTS Curated Protein Localization Evidence Scores 2025 cellular components containing KIFBP protein from the COMPARTMENTS Curated Protein Localization Evidence Scores 2025 dataset.
COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025 cellular components co-occuring with KIFBP protein in abstracts of biomedical publications from the COMPARTMENTS Text-mining Protein Localization Evidence Scores 2025 dataset.
DepMap CRISPR Gene Dependency cell lines with fitness changed by KIFBP gene knockdown relative to other cell lines from the DepMap CRISPR Gene Dependency dataset.
DISEASES Curated Gene-Disease Association Evidence Scores 2025 diseases involving KIFBP gene from the DISEASES Curated Gene-Disease Association Evidence Scores 2025 dataset.
DISEASES Experimental Gene-Disease Association Evidence Scores 2025 diseases associated with KIFBP gene in GWAS datasets from the DISEASES Experimental Gene-Disease Assocation Evidence Scores 2025 dataset.
DISEASES Text-mining Gene-Disease Association Evidence Scores 2025 diseases co-occuring with KIFBP gene in abstracts of biomedical publications from the DISEASES Text-mining Gene-Disease Assocation Evidence Scores 2025 dataset.
DisGeNET Gene-Disease Associations diseases associated with KIFBP gene in GWAS and other genetic association datasets from the DisGeNET Gene-Disease Associations dataset.
DisGeNET Gene-Phenotype Associations phenotypes associated with KIFBP gene in GWAS and other genetic association datasets from the DisGeNET Gene-Phenoptype Associations dataset.
GO Biological Process Annotations 2023 biological processes involving KIFBP gene from the curated GO Biological Process Annotations 2023 dataset.
GO Biological Process Annotations 2025 biological processes involving KIFBP gene from the curated GO Biological Process Annotations2025 dataset.
GO Molecular Function Annotations 2023 molecular functions performed by KIFBP gene from the curated GO Molecular Function Annotations 2023 dataset.
GO Molecular Function Annotations 2025 molecular functions performed by KIFBP gene from the curated GO Molecular Function Annotations 2025 dataset.
GTEx eQTL 2025 SNPs regulating expression of KIFBP gene from the GTEx eQTL 2025 dataset.
GTEx Tissue Gene Expression Profiles 2023 tissues with high or low expression of KIFBP gene relative to other tissues from the GTEx Tissue Gene Expression Profiles 2023 dataset.
GTEx Tissue-Specific Aging Signatures tissue samples with high or low expression of KIFBP gene relative to other tissue samples from the GTEx Tissue-Specific Aging Signatures dataset.
IMPC Knockout Mouse Phenotypes phenotypes of mice caused by KIFBP gene knockout from the IMPC Knockout Mouse Phenotypes dataset.
JASPAR Predicted Human Transcription Factor Targets 2025 transcription factors regulating expression of KIFBP gene predicted using known transcription factor binding site motifs from the JASPAR Predicted Human Transcription Factor Targets dataset.
JASPAR Predicted Mouse Transcription Factor Targets 2025 transcription factors regulating expression of KIFBP gene predicted using known transcription factor binding site motifs from the JASPAR Predicted Mouse Transcription Factor Targets 2025 dataset.
LINCS L1000 CMAP Chemical Perturbation Consensus Signatures small molecule perturbations changing expression of KIFBP gene from the LINCS L1000 CMAP Chemical Perturbations Consensus Signatures dataset.
LINCS L1000 CMAP CRISPR Knockout Consensus Signatures gene perturbations changing expression of KIFBP gene from the LINCS L1000 CMAP CRISPR Knockout Consensus Signatures dataset.
MGI Mouse Phenotype Associations 2023 phenotypes of transgenic mice caused by KIFBP gene mutations from the MGI Mouse Phenotype Associations 2023 dataset.
MoTrPAC Rat Endurance Exercise Training tissue samples with high or low expression of KIFBP gene relative to other tissue samples from the MoTrPAC Rat Endurance Exercise Training dataset.
PFOCR Pathway Figure Associations 2024 pathways involving KIFBP protein from the Wikipathways PFOCR 2024 dataset.
Replogle et al., Cell, 2022 K562 Essential Perturb-seq Gene Perturbation Signatures gene perturbations changing expression of KIFBP gene from the Replogle et al., Cell, 2022 K562 Essential Perturb-seq Gene Perturbation Signatures dataset.
Replogle et al., Cell, 2022 K562 Genome-wide Perturb-seq Gene Perturbation Signatures gene perturbations changing expression of KIFBP gene from the Replogle et al., Cell, 2022 K562 Genome-wide Perturb-seq Gene Perturbation Signatures dataset.
RummaGEO Drug Perturbation Signatures drug perturbations changing expression of KIFBP gene from the RummaGEO Drug Perturbation Signatures dataset.
RummaGEO Gene Perturbation Signatures gene perturbations changing expression of KIFBP gene from the RummaGEO Gene Perturbation Signatures dataset.
Sci-Plex Drug Perturbation Signatures drug perturbations changing expression of KIFBP gene from the Sci-Plex Drug Perturbation Signatures dataset.
TISSUES Curated Tissue Protein Expression Evidence Scores 2025 tissues with high expression of KIFBP protein from the TISSUES Curated Tissue Protein Expression Evidence Scores 2025 dataset.
TISSUES Experimental Tissue Protein Expression Evidence Scores 2025 tissues with high expression of KIFBP protein in proteomics datasets from the TISSUES Experimental Tissue Protein Expression Evidence Scores 2025 dataset.
TISSUES Text-mining Tissue Protein Expression Evidence Scores 2025 tissues co-occuring with KIFBP protein in abstracts of biomedical publications from the TISSUES Text-mining Tissue Protein Expression Evidence Scores 2025 dataset.