ERK pathway in Huntington's disease (WP3853)

Homo sapiens

This ERK pathway is implicated in Huntington's disease. The ligands BDNF, EGF, and Glu bind to their respective receptors (TrkB, EGFR, mGluR) and start a signal transduction pathway starting with RAS and RAF1, which leads to the stimulation of MEK then ERK. ERK promotes function of MSK1 (a downstream kinase), ElK1 and CREB (transcription factors), and caspases 3/7 (apoptotic molecules). These downstream targets are implicated in producing the effects of Huntington's disease at the cellular level. This pathway is based on Figure 1 from Bodai et al. Proteins on this pathway have targeted assays available via the [ CPTAC Assay Portal]


AAR&Co , Martina Summer-Kutmon , Kristina Hanspers , Andika Tan , Nirupama Benis , Eric Weitz , and Ash Iyer


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Homo sapiens


Diseases Rare Diseases


Pathway Ontology

signaling pathway disease pathway Huntington's disease pathway

Disease Ontology

Huntington's disease


Label Type Compact URI Comment
Glutamate Metabolite hmdb:HMDB0000148
CREB GeneProduct ensembl:ENSG00000118260
CASP7 GeneProduct ensembl:ENSG00000165806
RAS GeneProduct ensembl:ENSG00000174775
CASP3 GeneProduct ensembl:ENSG00000164305
NTRK2 GeneProduct ensembl:ENSG00000148053
MSK1 GeneProduct ensembl:ENSG00000100784
GRM1 GeneProduct ensembl:ENSG00000152822
ELK1 GeneProduct ensembl:ENSG00000126767
MAPK1 GeneProduct ensembl:ENSG00000100030
BDNF GeneProduct ensembl:ENSG00000176697
HTT GeneProduct ensembl:ENSG00000197386 mutant Huntingtin
EGF GeneProduct ensembl:ENSG00000138798
EGFR GeneProduct ensembl:ENSG00000146648
RAF1 GeneProduct ensembl:ENSG00000132155
MAP2K1 GeneProduct hgnc.symbol:MAP2K1
MAP2K2 GeneProduct hgnc.symbol:MAP2K2
MAPK3 GeneProduct hgnc.symbol:MAPK3


  1. A novel target for Huntington’s disease: ERK at the crossroads of signaling. The ERK signaling pathway is implicated in Huntington’s disease and its upregulation ameliorates pathology. Bodai L, Marsh JL. Bioessays. 2012 Feb;34(2):142–8. PubMed Europe PMC Scholia