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C57BL/6JCya-Httem1/Cya
Common Name:
Htt-KO
Product ID:
S-KO-02431
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Htt-KO
Strain ID
KOCMP-15194-Htt-B6J-VA
Gene Name
Htt
Product ID
S-KO-02431
Gene Alias
C430023I11Rik; Hd; Hdh; IT15
Background
C57BL/6JCya
NCBI ID
15194
Modification
Conventional knockout
Chromosome
5
Phenotype
MGI:96067
Document
Click here to download >>
Application
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Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Httem1/Cya mice (Catalog S-KO-02431) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000080036
NCBI RefSeq
NM_010414
Target Region
Exon 4~5
Size of Effective Region
~2.7 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Htt, short for huntingtin, is encoded by the HTT gene. An abnormal expansion of a glutamine stretch (polyQ) in its N-terminal sequence leads to Huntington's disease (HD), a devastating neurodegenerative disorder [1]. HTT is ubiquitous at both tissue and subcellular levels, interacting with many partners, yet its cellular function has long been elusive [1].

HD is caused by a CAG repeat expansion in exon 1 of the HTT gene, which is translated into a polyglutamine stretch in the huntingtin protein [2]. In YAC128 mice transgenic for human HTT with an expanded CAG repeat, an exon 1 only transcript (HTT1a) was detected, which is translated to produce the highly aggregation-prone and pathogenic exon 1 HTT protein, and its levels correlated with HTT aggregation [2]. In HD knock-in mice expressing full-length mutant HTT, the numbers of astrocytes and oligodendrocytes were not significantly altered, but myelin protein expression and myelin thickness of axons were reduced at 3 months of age, suggesting cytoplasmic mutant HTT mediates myelination defects in early disease stages without impacting glial cell differentiation and maturation [3].

In conclusion, the study of Htt through mouse models like YAC128 and HD knock-in mice has revealed that mutant Htt is closely associated with the pathogenesis of Huntington's disease, especially in aspects such as protein aggregation and myelination defects. Understanding the function of Htt and the impact of its mutation is crucial for developing therapies for HD.

References:

1. Saudou, Frédéric, Humbert, Sandrine. . The Biology of Huntingtin. In Neuron, 89, 910-26. doi:10.1016/j.neuron.2016.02.003. https://pubmed.ncbi.nlm.nih.gov/26938440/

2. Fienko, Sandra, Landles, Christian, Sathasivam, Kirupa, Kordasiewicz, Holly B, Bates, Gillian P. . Alternative processing of human HTT mRNA with implications for Huntington's disease therapeutics. In Brain : a journal of neurology, 145, 4409-4424. doi:10.1093/brain/awac241. https://pubmed.ncbi.nlm.nih.gov/35793238/

3. Yang, Sitong, Ma, Jingjing, Zhang, Han, Li, Xiao-Jiang, Guo, Xiangyu. 2023. Mutant HTT does not affect glial development but impairs myelination in the early disease stage. In Frontiers in neuroscience, 17, 1238306. doi:10.3389/fnins.2023.1238306. https://pubmed.ncbi.nlm.nih.gov/37539389/

Quality Control Standard
Sperm Test

Pre-cryopreservation: Measurement of sperm concentration, determination of sperm viability.

Post-cryopreservation: A vial of cryopreserved sperms is selected for in-vitro fertilization from each batch.

Environmental Standards:SPF
Available Region:Global
Source:Cyagen
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