BMPR1B

Chr 4

bone morphogenetic protein receptor type 1B

Also known as: ALK-6, ALK6, AMD3, AMDD, BDA1D, BDA2, CDw293

This gene encodes a member of the bone morphogenetic protein (BMP) receptor family of transmembrane serine/threonine kinases. The ligands of this receptor are BMPs, which are members of the TGF-beta superfamily. BMPs are involved in endochondral bone formation and embryogenesis. These proteins transduce their signals through the formation of heteromeric complexes of 2 different types of serine (threonine) kinase receptors: type I receptors of about 50-55 kD and type II receptors of about 70-80 kD. Type II receptors bind ligands in the absence of type I receptors, but they require their respective type I receptors for signaling, whereas type I receptors require their respective type II receptors for ligand binding. Mutations in this gene have been associated with primary pulmonary hypertension. Several transcript variants encoding two different isoforms have been found for this gene. [provided by RefSeq, Feb 2012]

ResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtAcromesomelic dysplasia 3
UniProtBrachydactyly A2
UniProtBrachydactyly A1, D

Clinical highlights

Gene-disease validity (ClinGen)
pulmonary arterial hypertension · UDDisputedevidence questions this relationship
Interpreting a novel variant
Loss of function is the curated mechanism (Gene2Phenotype) and the gene is intolerant of it in the population — truncating, frameshift and canonical splice variants carry more prior weight here than missense.Curated gene-level mechanism — a prior for triage, not a per-variant call.
0
Active trials
57
Pubs (1 yr)
P/LP submissions
P/LP missense
0.23
LOEUF· LoF intol.
LOF
Mechanism· G2P
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint?
0.23LOEUF
pLI 0.999
Z-score 4.55
OE 0.07 (0.030.23)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint?
0.27Z-score
OE missense 0.96 (0.861.05)
278 obs / 291.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.07 (0.030.23)
00.351.4
Missense OE?0.96 (0.861.05)
00.61.4
Synonymous OE?1.09
01.21.6
LoF obs/exp: 2 / 28.0Missense obs/exp: 278 / 291.0Syn Z: -0.68

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

BMPR1B · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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