Extensive Expression Analysis of Htt Transcripts in Brain Regions from the zQ175 HD Mouse Model Using a QuantiGene Multiplex Assay.

Extensive Expression Analysis of Htt Transcripts in Brain Regions from the zQ175 HD Mouse Model Using a QuantiGene Multiplex Assay.

Publication date: Nov 06, 2019

Huntington’s disease (HD) is an inherited neurodegenerative disorder caused by a CAG repeat expansion within exon 1 of the huntingtin (HTT) gene. HTT mRNA contains 67 exons and does not always splice between exon 1 and exon 2 leading to the production of a small polyadenylated HTTexon1 transcript, and the full-length HTT mRNA has three 3’UTR isoforms. We have developed a QuantiGene multiplex panel for the simultaneous detection of all of these mouse Htt transcripts directly from tissue lysates and demonstrate that this can replace the more work-intensive Taqman qPCR assays. We have applied this to the analysis of brain regions from the zQ175 HD mouse model and wild type littermates at two months of age. We show that the incomplete splicing of Htt occurs throughout the brain and confirm that this originates from the mutant and not endogenous Htt allele. Given that HTTexon1 encodes the highly pathogenic exon 1 HTT protein, it is essential that the levels of all Htt transcripts can be monitored when evaluating HTT lowering approaches. Our QuantiGene panel will allow the rapid comparative assessment of all Htt transcripts in cell lysates and mouse tissues without the need to first extract RNA.

Open Access PDF

Papadopoulou, A.S., Gomez-Paredes, C., Mason, M.A., Taxy, B.A., Howland, D., and Bates, G.P. Extensive Expression Analysis of Htt Transcripts in Brain Regions from the zQ175 HD Mouse Model Using a QuantiGene Multiplex Assay. 06750. 2019 Sci Rep (9):1.

Concepts Keywords
Allele Neurodegenerative disorder
Brain Nucleic acids
Endogenous Branches of biology
Exon Spliceosome
Exons Gene expression
Gene RNA splicing
Huntingtin Molecular biology
Huntington Exon
Isoforms Messenger RNA
MRNA Huntingtin
Multiplex HTT
Mutant
Neurodegenerative Disorder
Pathogenic
Polyadenylated
Protein
QPCR
RNA
Sci
Splicing

Semantics

Type Source Name
gene UNIPROT HTT
disease MESH neurodegenerative disorder
gene UNIPROT SLC6A4
drug DRUGBANK Coenzyme M
disease MESH movement disorders
disease MESH Dementia
disease DOID Dementia
disease MESH development
drug DRUGBANK Zinc
gene UNIPROT EHD1
gene UNIPROT GOPC
gene UNIPROT DEPP1
gene UNIPROT PAGR1
disease MESH tic
gene UNIPROT PSD4
gene UNIPROT SET
gene UNIPROT UBC
gene UNIPROT EIF4A2
disease MESH heterozygotes
gene UNIPROT LUM
gene UNIPROT PPIB
gene UNIPROT RPL13A
gene UNIPROT CANX
gene UNIPROT ATP5F1B
drug DRUGBANK ATP
disease MESH **p
drug DRUGBANK Proline
gene UNIPROT OCA2
gene UNIPROT SERPINA3
gene UNIPROT FHL5
drug DRUGBANK Water
gene UNIPROT TNFSF14
drug DRUGBANK Nitrogen
drug DRUGBANK Tromethamine
gene UNIPROT PRDM10
drug DRUGBANK Sodium lauryl sulfate
gene UNIPROT SDS
drug DRUGBANK Edetic Acid
gene UNIPROT ERVK-9
gene UNIPROT ERVK-7
gene UNIPROT ERVK-6
gene UNIPROT ERVK-8
gene UNIPROT ERVK-24
gene UNIPROT ERVK-25
gene UNIPROT ERVK-19
gene UNIPROT HERVK_113
gene UNIPROT ERVK-10
gene UNIPROT HERV-K104
gene UNIPROT ERVK-18
gene UNIPROT ERVK-21
gene UNIPROT TUBE1
gene UNIPROT CASP1
gene UNIPROT CES2
drug DRUGBANK Ethanol
drug DRUGBANK Medical air
gene UNIPROT RIOX2
gene UNIPROT MNT
gene UNIPROT RIOX1
drug DRUGBANK Aspartame
gene UNIPROT KIT
gene UNIPROT PELI1
disease MESH Huntington disease
disease DOID Huntington disease
gene UNIPROT CCAR1
gene UNIPROT PROC
pathway BSID mRNA Splicing
gene UNIPROT EXOG
gene UNIPROT SCN8A
pathway BSID Signal amplification
gene UNIPROT CYREN
gene UNIPROT FSCN1
gene UNIPROT FAN1
pathway BSID Reproduction

Original Article

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