RGD Reference Report - Diminished hippocalcin expression in Huntington's disease brain does not account for increased striatal neuron vulnerability as assessed in primary neurons.

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Diminished hippocalcin expression in Huntington's disease brain does not account for increased striatal neuron vulnerability as assessed in primary neurons.
Authors:	Rudinskiy, N Kaneko, YA Beesen, AA Gokce, O Regulier, E Deglon, N Luthi-Carter, R
Citation:	Rudinskiy N, etal., J Neurochem. 2009 Oct;111(2):460-72. doi: 10.1111/j.1471-4159.2009.06344.x. Epub 2009 Aug 17.
RGD ID:	9693681
Pubmed:	PMID:19686238 (View Abstract at PubMed)
DOI:	DOI:10.1111/j.1471-4159.2009.06344.x (Journal Full-text)
Hippocalcin is a neuronal calcium sensor protein previously implicated in regulating neuronal viability and plasticity. Hippocalcin is the most highly expressed neuronal calcium sensor in the medium spiny striatal output neurons that degenerate selectively in Huntington's disease (HD). We have previously shown that decreased hippocalcin expression occurs in parallel with the onset of disease phenotype in mouse models of HD. Here we show by in situ hybridization histochemistry that hippocalcin RNA is also diminished by 63% in human HD brain. These findings lead us to hypothesize that diminished hippocalcin expression might contribute to striatal neurodegeneration in HD. We tested this hypothesis by assessing whether restoration of hippocalcin expression would decrease striatal neurodegeneration in cellular models of HD comprising primary striatal neurons exposed to mutant huntingtin, the mitochondrial toxin 3-nitropropionic acid or an excitotoxic concentration of glutamate. Counter to our hypothesis, hippocalcin expression did not improve the survival of striatal neurons under these conditions. Likewise, expression of hippocalcin together with interactor proteins including the neuronal apoptosis inhibitory protein did not increase the survival of striatal cells in cellular models of HD. These results indicate that diminished hippocalcin expression does not contribute to HD-related neurodegeneration.

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Term	Qualifier	Evidence	With	Reference	Notes	Source	Original Reference(s)
Huntington's disease		IEP		9693681	mRNA:decreased expression:brain (human)	RGD
Huntington's disease		ISO	HPCA (Homo sapiens)	9693681; 9693681	mRNA:decreased expression:brain (human)	RGD

Objects Annotated

Genes (Rattus norvegicus)

Hpca (hippocalcin)

Genes (Mus musculus)

Hpca (hippocalcin)

Genes (Homo sapiens)

HPCA (hippocalcin)

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Diminished hippocalcin expression in Huntington's disease brain does not account for increased striatal neuron vulnerability as assessed in primary neurons.