RGD Reference Report - Lymphopenia in Ednrb-deficient rat was strongly modified by genetic background. - Rat Genome Database

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Lymphopenia in Ednrb-deficient rat was strongly modified by genetic background.

Authors: Dang, R  Sasaki, N  Nishino, T  Nakanishi, M  Torigoe, D  Agui, T 
Citation: Dang R, etal., Biomed Res. 2012;33(4):249-53.
RGD ID: 7207471
Pubmed: PMID:22975636   (View Abstract at PubMed)

The endothelin signaling pathway plays an important role in the migration, proliferation, and differentiation of neural crest cells. Mutations in the gene encoding the endothelin receptor type B (EDNRB) cause three symptoms: aganglionosis, pigmented disorder and hearing loss. In addition, the Ednrb null mice show abnormal splenic microarchitecture with lymphopenia. In this study, we examined whether similar phenotypes are reproduced in three Ednrb-null rat strains that we established previously. AGH-Ednrb(sl)/(sl) strain showed a low white blood cell count, significant size reduction and abnormal microarchitecture of spleen. Thymus displayed a marked reduction in the size, but maintained a normal CD4/CD8 ratio. In contrast, splenic cellularity was reduced to < 15%, and splenic B and T cell numbers were reduced, showing a splenic lymphopenia. Interestingly, Ednrb-null rats in the LE and F344 genetic background did not show these abnormalities. These data show that proper T and B cell development is dependent on the endothelin signaling pathway, however, modifier gene(s) might be differentially expressed in these strain to modulate or compensate for the effect of the Ednrb deficiency.

RGD Manual Disease Annotations    Click to see Annotation Detail View

Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
AR-Ednrbsl/HkvRatlymphopenia  IAGP  RGD 
EDNRBHumanlymphopenia  ISOEdnrb (Rattus norvegicus) RGD 
EdnrbRatlymphopenia  IAGP  RGD 
EdnrbMouselymphopenia  ISOEdnrb (Rattus norvegicus) RGD 
EdnrbslRatlymphopenia  IAGP  RGD 

Objects Annotated

Genes (Rattus norvegicus)
Ednrb  (endothelin receptor type B)
Ednrbsl  (endothelin receptor type B, spotting lethal)

Genes (Mus musculus)
Ednrb  (endothelin receptor type B)

Genes (Homo sapiens)
EDNRB  (endothelin receptor type B)

AR-Ednrbsl/Hkv  (Aganglionosis rat)

Additional Information