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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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IGBP1
immunoglobulin binding protein 1
Chromosome X · Xq13.1
NCBI Gene: 3476Ensembl: ENSG00000089289.17HGNC: HGNC:5461UniProt: P78318
101PubMed Papers
21Diseases
0Drugs
1Pathogenic Variants
FUNCTIONAL ROLE
Highly Constrained
CLINICAL
OMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingprotein phosphatase regulator activitynegative regulation of transcription by RNA polymerase IInegative regulation of stress-activated MAPK cascadecorpus callosum agenesis-intellectual disability-coloboma-micrognathia syndromeAgenesis of the corpus callosum - intellectual disability - coloboma - micrognathianeurodegenerative diseasenasopharyngeal carcinoma
✦AI Summary

IGBP1 (immunoglobulin binding protein 1) is an X-linked phosphoprotein associated with the B cell receptor complex that plays multiple roles in signal transduction and protein regulation 1. The protein is structurally related to yeast TAP42, a component of the rapamycin-sensitive signaling pathway 2, and regulates phosphatase activity by protecting partially folded catalytic subunits of PP2A, PP4, and PP6 from degradation until association with regulatory subunits. IGBP1 influences B cell differentiation, proliferation, apoptosis, and tolerance 1. Clinically, IGBP1 serves as a biomarker for lupus nephritis (LN) activity. Serum IGBP1 levels >547.45 ng/mL show 93.8% sensitivity and 96.9% specificity for detecting active renal disease 3, correlating with proteinuria and renal disease activity indices 1. Elevated plasma IGBP1 predicts future LN development in SLE patients without nephritis 4. IGBP1 expression is enriched in heart failure patients treated with SGLT2 inhibitors, supporting autophagy and mitochondrial health 5. An IGBP1 lncRNA variant (IGBP1-AS1) promotes nasopharyngeal carcinoma progression via miR-150-5p regulation 6. Mutations in IGBP1 associate with X-linked intellectual developmental disorder 7, though further validation is warranted.

Sources cited
1
IGBP1 is a phosphoprotein associated with B cell receptor complex; regulates B cell differentiation, proliferation, apoptosis, and tolerance
PMID: 31137925
2
Human IGBP1 (alpha 4) gene structure is closely related to yeast TAP42 protein of rapamycin-sensitive signal transduction pathway; localized to chromosome Xq13.1-q13.3
PMID: 9441740
3
Serum IGBP1 level of 547.45 ng/mL is a valid biomarker for detecting active lupus nephritis with 93.8% sensitivity and 96.9% specificity
PMID: 36418233
4
Urine IGBP1 levels elevated in lupus nephritis patients and correlate with clinical activity indices and histological activity index
PMID: 31137925
5
Plasma IGBP1 predicts future development of lupus nephritis in SLE patients without nephritis; when combined with anti-dsDNA antibody shows 97% specificity
PMID: 32183589
6
IGBP1 is among proteins enriched by SGLT2 inhibitors that promote autophagy and cellular iron mobilization in heart failure patients
PMID: 39217550
7
LncRNA IGBP1-AS1 targets miR-150-5p to increase ZEB1 expression and promote nasopharyngeal carcinoma progression
PMID: 35402187
8
IGBP1 implicated in X-linked intellectual developmental disorder; replication studies warranted
PMID: 23871722
Disease Associationsⓘ21
corpus callosum agenesis-intellectual disability-coloboma-micrognathia syndromeOpen Targets
0.54Moderate
Agenesis of the corpus callosum - intellectual disability - coloboma - micrognathiaOpen Targets
0.49Moderate
neurodegenerative diseaseOpen Targets
0.26Weak
nasopharyngeal carcinomaOpen Targets
0.08Suggestive
breast cancerOpen Targets
0.07Suggestive
lobular neoplasiaOpen Targets
0.05Suggestive
hepatocellular carcinomaOpen Targets
0.05Suggestive
esophageal squamous cell carcinomaOpen Targets
0.05Suggestive
systemic lupus erythematosusOpen Targets
0.05Suggestive
neoplasmOpen Targets
0.04Suggestive
lung adenocarcinomaOpen Targets
0.03Suggestive
cancerOpen Targets
0.03Suggestive
allergic asthmaOpen Targets
0.03Suggestive
nephritisOpen Targets
0.03Suggestive
chronic asthmaOpen Targets
0.03Suggestive
asthmaOpen Targets
0.02Suggestive
rheumatoid arthritisOpen Targets
0.02Suggestive
acute lymphoblastic leukemiaOpen Targets
0.02Suggestive
Opitz G/BBB syndromeOpen Targets
0.02Suggestive
infectionOpen Targets
0.02Suggestive
Intellectual developmental disorder, X-linked, syndromic 28UniProt
Pathogenic Variants1
NM_001551.3(IGBP1):c.-57_-55delinsAAPathogenic
Corpus callosum agenesis-intellectual disability-coloboma-micrognathia syndrome
☆☆☆☆2003
View on ClinVar ↗
Related Genes
ITGA4Protein interaction100%AKT1S1Protein interaction100%RPTORProtein interaction100%MTORProtein interaction100%MLST8Protein interaction100%ITGB1Protein interaction100%
Tissue Expression6 tissues
Ovary
100%
Brain
62%
Heart
61%
Lung
49%
Bone Marrow
45%
Liver
21%
Gene Interaction Network
Click a node to explore
IGBP1ITGA4AKT1S1RPTORMTORMLST8ITGB1
PROTEIN STRUCTURE
Preparing viewer…
PDB4IYP · 2.80 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.24Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.08 [0.03–0.24]
RankingsWhere IGBP1 stands among ~20K protein-coding genes
  • #4,722of 20,598
    Most Researched101 · top quartile
  • #4,880of 5,498
    Most Pathogenic Variants1
  • #726of 17,882
    Most Constrained (LOEUF)0.24 · top 5%
Genes detectedIGBP1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Reaffirmation of Mechanistic Proteomic Signatures Accompanying SGLT2 Inhibition in Patients With Heart Failure: A Validation Cohort of the EMPEROR Program.
PMID: 39217550
J Am Coll Cardiol · 2024
1.00
2
Validity of immunoglobulin-binding protein 1 as a biomarker for lupus nephritis.
PMID: 36418233
Lupus · 2023
0.90
3
Expression and chromosomal localization of the human alpha 4/IGBP1 gene, the structure of which is closely related to the yeast TAP42 protein of the rapamycin-sensitive signal transduction pathway.
PMID: 9441740
Genomics · 1997
0.80
4
Immunoglobulin Binding Protein 1 as a Potential Urine Biomarker in Patients with Lupus Nephritis.
PMID: 31137925
Int J Mol Sci · 2019
0.70
5
Plasma immunoglobulin binding protein 1 as a predictor of development of lupus nephritis.
PMID: 32183589
Lupus · 2020
0.60