Huntingtin-interacting protein-2 (Hip-2) was identified as a human protein
specifically associated with huntingtin in vitro. a gene product affected i
n patients with Huntington disease (HD). It is a ubiquitin-conjugating enzy
me identical to the previously characterized bovine E2-25k. We identified t
he mouse Hip-2 homologue (mHip-2) and examined its distribution patterns in
the developing mouse brain ill order to gain an insight into the functiona
l significance of the Hip-2 protein in the normal brain as well as in the p
athogenesis of HD. As reported with huntingtin, the mHip-2 mRNA expression
developed in parallel with neuronal maturation and became distributed widel
y in the postnatal mouse brain. This spatiotemporal pattern of mHip-2 mRNA
expression resembled that of huntingtin. We further demonstrated that mHip-
2 mRNA was colocalized with huntingtin-like immunoreactivity in a single ne
uron. These findings suggested that the Hip-2 interacted with huntingtin in
vivo and played an important role in HD pathogenesis. (C) 1999 Elsevier Sc
ience B.V. All rights reserved.