GH-LCR

Chr 17ARAD

growth hormone locus control region

This element represents a locus control region (LCR) that can confer copy number-dependent, position-independent expression on members of the human growth hormone gene cluster in transgenic assays. It regulates expression of the GH1 (growth hormone 1), CSHL1 (chorionic somatomammotropin hormone-like 1), CSH1 (chorionic somatomammotropin hormone 1 (placental lactogen)), GH2 (growth hormone 2) and CSH2 (chorionic somatomammotropin hormone 2) genes in this cluster. This LCR spans approximately 40 kb upstream of the GH1 gene and is characterized by five major DNase I hypersensitive sites (HSI-HSV). It overlaps the CD79B (CD79b molecule, immunoglobulin-associated beta) gene, and also the 3' end of the SCN4A (sodium channel, voltage gated, type IV alpha subunit) gene. This LCR includes several transcription factor binding sites as well as subregions with enhancer, enhancer-blocking and boundary element activity. Three subregions were shown to be active enhancers by ChIP-STARR-seq in naive human embryonic stem cells, where all are marked by the H3K4me1 histone modification and one is additionally marked by H3K27ac. A subregion was also validated as a functional repressive element by Sharpr-MPRA (Systematic high-resolution activation and repression profiling with reporter tiling using massively parallel reporter assays) in HepG2 liver carcinoma cells (group: HepG2 Repressive non-DNase unmatched - State 20:ReprD, Polycomb repression w. Duke DNase/promoter and conservation enriched). This locus also includes seven accessible chromatin subregions, six of which were validated as enhancers and one as a silencer, based on their ability to activate or repress an origin of replication minimal core promoter by the ATAC-STARR-seq (assay for transposase-accessible chromatin with self-transcribing active regulatory region sequencing) MPRA in GM12878 lymphoblastoid cells. Polymorphisms in this LCR may be associated with isolated growth hormone deficiency (IGHD), affecting serum IGF-I levels and height. [provided by RefSeq, May 2023]

Primary Disease Associations & Inheritance

Growth hormone deficiency, isolated, type IAMIM #262400
AR
Growth hormone deficiency, isolated, type IBMIM #612781
Growth hormone deficiency, isolated, type IIMIM #173100
AD
Kowarski syndromeMIM #262650
AR
1
Active trials
20
Pathogenic / LP
486
ClinVar variants
1
Pubs (1 yr)
Missense Z
LOEUF
Clinical SummaryGH-LCR
📋
ClinVar Variants
20 Pathogenic / Likely Pathogenic· 271 VUS of 486 total submissions
💊
Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available
Some data sources returned errors (2)

ensembl: Error: Ensembl fetch failed: 400 for https://rest.ensembl.org/lookup/symbol/homo_sapiens/GH-LCR?content-type=application/json&expand=1

gnomad: Error: Gene not found

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

486 submitted variants in ClinVar

Classification Summary

Pathogenic3
Likely Pathogenic17
VUS271
Likely Benign148
Benign44
Conflicting3
3
Pathogenic
17
Likely Pathogenic
271
VUS
148
Likely Benign
44
Benign
3
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
3
0
0
0
3
Likely Pathogenic
8
6
3
0
17
VUS
3
242
21
5
271
Likely Benign
0
3
57
88
148
Benign
0
0
44
0
44
Conflicting
3
Total1425112593486

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

GH-LCR · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Literature
Landmark / reviewRecent case evidence
Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

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