HomeAboutRankingsData Sources
© 2026 GeneE
🧬
GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
ⓘGeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
RHBG
Rh family B glycoprotein
Chromosome 1 · 1q22
NCBI Gene: 57127Ensembl: ENSG00000132677.14HGNC: HGNC:14572UniProt: Q9H310
36PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transporter
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
spectrin-associated cytoskeletonankyrin bindingcarbon dioxide transmembrane transporter activitybasolateral plasma membranefunctional neutrophil defectsystemic inflammatory response syndromeHyperammonemiaisolated asymptomatic elevation of creatine phosphokinase
✦AI Summary

RHBG (Rh family B glycoprotein) is a mammalian ammonium transporter that mediates transepithelial ammonia handling and acid-base homeostasis 1. The protein functions primarily as an electrogenic NH4+ transporter, though it can transport both NH4+ and NH3 species across basolateral plasma membranes of epithelial cells 2. Beyond ammonia transport, RHBG exhibits dual functionality as a transceptor—it also transports CO2 and initiates intracellular signaling through MyD88-dependent NFκB activation in response to ammonia, independent of canonical toll-like receptor mechanisms 23. In the kidney, RHBG localizes to α-intercalated cells of the collecting duct via tyrosine-based sorting signals and ankyrin-G anchoring 4. Notably, while RHBG is expressed in rodent kidneys and skeletal muscle 5, it is not detectable in normal human kidney tissue, where RhCG serves as the primary ammonia transporter 6. RHBG expression in cancer cells is regulated by Wnt/β-catenin-TCF4 signaling 7, linking it to tumorigenesis. The protein's dual transport-signaling properties suggest distinct therapeutic targets for managing hyperammonemia and potentially modulating cancer cell metabolism.

Sources cited
1
RHBG transports both CO2 and NH3 and contributes to renal acid secretion
PMID: 24077989
2
RHBG acts as a transceptor activating MyD88-dependent NFκB signaling in response to ammonia
PMID: 39052834
3
RHBG physically interacts with carbonic anhydrase-IV with modulatory effects on ammonia transport
PMID: 40811810
4
RHBG expression is regulated by Wnt/β-catenin-TCF4 signaling in cancer cells
PMID: 26029888
5
RHBG is not detectable in human kidney while RhCG is the major ammonia transporter
PMID: 19357182
6
RHBG is expressed in mouse skeletal muscle, particularly in slow-twitch fibers
PMID: 26471760
7
RHBG is a specific mammalian ammonium transporter involved in ammonia homeostasis
PMID: 17106214
8
RHBG requires tyrosine-based sorting signals and ankyrin-G for basolateral membrane targeting
PMID: 15611082
Disease Associationsⓘ20
functional neutrophil defectOpen Targets
0.33Weak
systemic inflammatory response syndromeOpen Targets
0.08Suggestive
HyperammonemiaOpen Targets
0.07Suggestive
isolated asymptomatic elevation of creatine phosphokinaseOpen Targets
0.05Suggestive
non-small cell lung carcinomaOpen Targets
0.05Suggestive
isolated hyperferritinemiaOpen Targets
0.05Suggestive
FTH1-related iron overloadOpen Targets
0.05Suggestive
hemochromatosis type 5Open Targets
0.05Suggestive
hypoparathyroidism, familial isolated, 2Open Targets
0.04Suggestive
hepatocellular carcinomaOpen Targets
0.04Suggestive
pseudohyperaldosteronism type 2Open Targets
0.03Suggestive
Liddle syndrome 2Open Targets
0.03Suggestive
Liddle syndrome 3Open Targets
0.03Suggestive
hereditary hyperferritinemia with congenital cataractsOpen Targets
0.03Suggestive
Liddle syndrome 1Open Targets
0.03Suggestive
Barrett's esophagusOpen Targets
0.03Suggestive
chronic kidney diseaseOpen Targets
0.03Suggestive
colorectal cancerOpen Targets
0.02Suggestive
breast cancerOpen Targets
0.02Suggestive
cancerOpen Targets
0.02Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
RHAGShared pathway50%RHDShared pathway50%RHCEShared pathway50%RHCGShared pathway38%AQP6Shared pathway25%SLC12A1Shared pathway18%
Tissue Expression6 tissues
Ovary
100%
Liver
23%
Brain
1%
Lung
1%
Heart
0%
Bone Marrow
0%
Gene Interaction Network
Click a node to explore
RHBGRHAGRHDRHCERHCGAQP6SLC12A1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q9H310
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.16LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.89 [0.69–1.16]
RankingsWhere RHBG stands among ~20K protein-coding genes
  • #10,851of 20,598
    Most Researched36
  • #12,122of 17,882
    Most Constrained (LOEUF)1.16
Genes detectedRHBG
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Relative CO₂/NH₃ permeabilities of human RhAG, RhBG and RhCG.
PMID: 24077989
J Membr Biol · 2013
1.00
2
Ammonia transporter RhBG initiates downstream signaling and functional responses by activating NFκB.
PMID: 39052834
Proc Natl Acad Sci U S A · 2024
0.90
3
Rhbg interaction with CA-IV and its effects on NH
PMID: 40811810
Am J Physiol Cell Physiol · 2025
0.80
4
Wnt/β-Catenin Signaling Regulates the Expression of the Ammonium Permease Gene RHBG in Human Cancer Cells.
PMID: 26029888
PLoS One · 2015
0.70
5
RhCG is the major putative ammonia transporter expressed in the human kidney, and RhBG is not expressed at detectable levels.
PMID: 19357182
Am J Physiol Renal Physiol · 2009
0.60