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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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GCM2
glial cells missing transcription factor 2
Chromosome 6 Β· 6p24.2
NCBI Gene: 9247Ensembl: ENSG00000124827.7HGNC: HGNC:4198UniProt: O75603
48PubMed Papers
22Diseases
0Drugs
22Pathogenic Variants
FUNCTIONAL ROLE
Transcription Factor
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
transcription by RNA polymerase IIintracellular calcium ion homeostasisnucleusparathyroid gland developmenthypoparathyroidism, familial isolated, 2hyperparathyroidism 4Familial isolated hypoparathyroidismhyperparathyroidism
✦AI Summary

GCM2 (glial cells missing transcription factor 2) is a critical transcription factor essential for parathyroid gland development and function. GCM2 is expressed specifically in developing pharyngeal pouches and marks parathyroid-specific domains within the common thymus-parathyroid primordium before morphological distinctions are present 1. The protein functions by binding specific DNA sequences and activating gene transcription, including direct regulation of parathyroid hormone (PTH) expression through cooperation with other transcription factors like Gata3 and MafB 2. Single-cell analysis reveals GCM2 is highly expressed in parathyroid progenitor cells, with expression decreasing as cells mature into PTH-expressing epithelial cells 3. Loss-of-function GCM2 mutations cause familial isolated hypoparathyroidism, with affected individuals showing hypocalcemia and reduced PTH levels 4 5. Conversely, activating GCM2 variants are associated with familial isolated hyperparathyroidism and may contribute to more aggressive parathyroid disease, including parathyroid carcinoma 5 6. These variants show enhanced transcriptional activity and increased PTH promoter stimulation but have low penetrance in the general population 6. Understanding GCM2's role provides insight into parathyroid disorders and calcium homeostasis regulation 7.

Sources cited
1
GCM2 expression marks parathyroid-specific domains in pharyngeal pouches during early development
PMID: 11335122
2
GCM2 cooperates with Gata3 and MafB to activate PTH gene transcription
PMID: 25917456
3
Single-cell analysis shows GCM2 is highly expressed in parathyroid progenitors
PMID: 38977309
4
GCM2 mutations can cause isolated hypoparathyroidism
PMID: 18182452
5
Loss-of-function and activating GCM2 variants cause hypoparathyroidism and hyperparathyroidism respectively
PMID: 35038313
6
Activating GCM2 variants are associated with familial hyperparathyroidism but have low penetrance
PMID: 34967908
7
GCM2 is central to parathyroid development and understanding parathyroid disorders
PMID: 30390809
Disease Associationsβ“˜22
hypoparathyroidism, familial isolated, 2Open Targets
0.72Strong
hyperparathyroidism 4Open Targets
0.71Strong
Familial isolated hypoparathyroidismOpen Targets
0.61Moderate
hyperparathyroidismOpen Targets
0.58Moderate
neurodegenerative diseaseOpen Targets
0.53Moderate
familial hypoparathyroidismOpen Targets
0.49Moderate
parathyroid diseaseOpen Targets
0.48Moderate
hypoparathyroidism, familial isolated 1Open Targets
0.48Moderate
HypercalcemiaOpen Targets
0.43Moderate
familial isolated hyperparathyroidismOpen Targets
0.39Weak
familial isolated hypoparathyroidism due to agenesis of parathyroid glandOpen Targets
0.39Weak
endocrine system diseaseOpen Targets
0.33Weak
genetic disorderOpen Targets
0.19Weak
menopauseOpen Targets
0.19Weak
metabolic diseaseOpen Targets
0.11Weak
breast cancerOpen Targets
0.09Suggestive
pseudohypoparathyroidism type 2Open Targets
0.08Suggestive
ovulationOpen Targets
0.08Suggestive
Parathyroid Gland CarcinomaOpen Targets
0.06Suggestive
ovarian dysfunctionOpen Targets
0.06Suggestive
Hyperparathyroidism 4UniProt
Hypoparathyroidism, familial isolated, 2UniProt
Pathogenic Variants22
NM_004752.4(GCM2):c.139C>T (p.Arg47Cys)Pathogenic
not provided|Hyperparathyroidism 4;Hypoparathyroidism, familial isolated, 2|Hyperparathyroidism 4
β˜…β˜…β˜†β˜†2025β†’ Residue 47
NM_004752.4(GCM2):c.90+2T>GLikely pathogenic
Familial hypoparathyroidism|not provided
β˜…β˜…β˜†β˜†2025
NM_004752.4(GCM2):c.408C>A (p.Tyr136Ter)Pathogenic
Familial hypoparathyroidism|not provided
β˜…β˜…β˜†β˜†2024β†’ Residue 136
NM_004752.4(GCM2):c.199C>T (p.Arg67Cys)Likely pathogenic
GCM2-related disorder|not provided|Hyperparathyroidism 4;Hypoparathyroidism, familial isolated, 2
β˜…β˜…β˜†β˜†2024β†’ Residue 67
NM_004752.4(GCM2):c.1098C>A (p.Cys366Ter)Likely pathogenic
Hypoparathyroidism, familial isolated, 2
β˜…β˜†β˜†β˜†2024β†’ Residue 366
NM_004752.4(GCM2):c.1048C>T (p.Gln350Ter)Likely pathogenic
Hypoparathyroidism, familial isolated, 2;Hyperparathyroidism 4
β˜…β˜†β˜†β˜†2024β†’ Residue 350
NM_004752.4(GCM2):c.140G>T (p.Arg47Leu)Pathogenic
Hypoparathyroidism, familial isolated, 2|not provided
β˜…β˜†β˜†β˜†2024β†’ Residue 47
NM_004752.4(GCM2):c.1100_1103del (p.Arg367fs)Likely pathogenic
Hypoparathyroidism, familial isolated, 2;Hyperparathyroidism 4
β˜…β˜†β˜†β˜†2024β†’ Residue 367
NM_004752.4(GCM2):c.115C>T (p.Arg39Ter)Likely pathogenic
not provided
β˜…β˜†β˜†β˜†2024β†’ Residue 39
NM_004752.4(GCM2):c.199C>G (p.Arg67Gly)Likely pathogenic
Hypoparathyroidism, familial isolated, 2
β˜…β˜†β˜†β˜†2024β†’ Residue 67
NM_004752.4(GCM2):c.1216C>T (p.Arg406Ter)Likely pathogenic
Hypoparathyroidism, familial isolated, 2;Hyperparathyroidism 4
β˜…β˜†β˜†β˜†2024β†’ Residue 406
NM_004752.4(GCM2):c.22G>T (p.Glu8Ter)Pathogenic
Familial hypoparathyroidism
β˜…β˜†β˜†β˜†2023β†’ Residue 8
NM_004752.4(GCM2):c.1104C>A (p.Tyr368Ter)Pathogenic
not provided
β˜…β˜†β˜†β˜†2023β†’ Residue 368
NM_004752.4(GCM2):c.853del (p.Ser285fs)Likely pathogenic
Hypoparathyroidism, familial isolated, 2
β˜…β˜†β˜†β˜†2023β†’ Residue 285
NM_004752.4(GCM2):c.1A>G (p.Met1Val)Likely pathogenic
Hyperparathyroidism 4
β˜…β˜†β˜†β˜†2021β†’ Residue 1
NM_004752.4(GCM2):c.1504A>G (p.Asn502Asp)Likely pathogenic
not provided
β˜…β˜†β˜†β˜†2016β†’ Residue 502
NM_004752.4(GCM2):c.456+2dupPathogenic
not provided
β˜…β˜†β˜†β˜†2016
NC_000006.12:g.(?_10875891)_(10881865_?)delPathogenic
not provided
β˜…β˜†β˜†β˜†2016
NM_004752.4(GCM2):c.1148T>C (p.Ile383Thr)Likely pathogenic
Hyperparathyroidism 4;Hypoparathyroidism, familial isolated, 2
β˜…β˜†β˜†β˜†β†’ Residue 383
NM_004752.4(GCM2):c.1400del (p.Pro467fs)Pathogenic
Hypoparathyroidism, familial isolated, 2
β˜†β˜†β˜†β˜†2021β†’ Residue 467
View on ClinVar β†—
Related Genes
CASRProtein interaction95%PTHProtein interaction91%HSFY1Protein interaction79%GCM1Protein interaction73%FOXN1Protein interaction72%GLIS3Shared pathway25%
Tissue Expression6 tissues
Ovary
100%
Bone Marrow
33%
Brain
33%
Heart
0%
Lung
0%
Liver
0%
Gene Interaction Network
Click a node to explore
GCM2CASRPTHHSFY1GCM1FOXN1GLIS3
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted Β· UniProt O75603
View on AlphaFold β†—
Constraintβ“˜
LOEUFβ“˜
1.21LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.81 [0.56–1.21]
RankingsWhere GCM2 stands among ~20K protein-coding genes
  • #9,076of 20,598
    Most Researched48
  • #2,107of 5,498
    Most Pathogenic Variants22
  • #12,668of 17,882
    Most Constrained (LOEUF)1.21
Genes detectedGCM2
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Familial Hyperparathyroidism.
PMID: 33716975
Front Endocrinol (Lausanne) Β· 2021
1.00
2
Gata3 cooperates with Gcm2 and MafB to activate parathyroid hormone gene expression by interacting with SP1.
PMID: 25917456
Mol Cell Endocrinol Β· 2015
0.90
3
Analysis of the GCM2 gene in isolated hypoparathyroidism: a molecular and biochemical study.
PMID: 18182452
J Clin Endocrinol Metab Β· 2008
0.80
4
Novel Glial Cells Missing-2 (GCM2) variants in parathyroid disorders.
PMID: 35038313
Eur J Endocrinol Β· 2022
0.70
5
Single-cell analysis reveals transcriptional dynamics in healthy primary parathyroid tissue.
PMID: 38977309
Genome Res Β· 2024
0.60