Name of Resource

    Transgenic HD Minipig (TgHD, PIGMOD Center)

    Name of Principal Investigator - Title

    Prof

    Name of Principal Investigator - First name

    Jan

    Name of Principal Investigator - Last name

    Motlik

    Address of institution -Institution

    Institute of Animal Physiology and Genetics-PIGMOD Center

    Address of institution - Street address

    Rumburská 89

    Address of institution - City

    Liběchov

    Address of institution - Postcode

    27721

    Country

    Czech Republic

    Contact email
    Summary

    At present, we are probably the only facility to be breeding TgHD minipigs. We have four generations, around 180 animals. The animals are beiing studied for phenotype development, and also first preclinical testing with a pharmaceutical company Uniqure is beeing done. We hope to attract more companies for testing their therapeutic treatment on this unique large animal model for Huntingon's disease. Funded by CHDI, Czech-Norwegian Research Programme 7F14308, National Programme of Sustainibility LO1609

    Q1a. Please indicate below if your cohort includes or expects to include, incidence of the following conditions? (1)

    Huntington's disease

    Q1b. Does your resource hold

    Animals| Other - animal-derived embryonic neural stem cells

    Q2a. Does the resource act as a centre for access and distribution to external groups (who are not the Principal Investigators (PI) for the resource)?

    Yes

    Q2b. If Yes, what procedures and rules apply for access?

    Apply to PI or co-ordinator at resource|Access through collaboration with PI only|International access

    Q3a. Does your resource develop experimental models (animal/cell) for external groups?

    Yes

    Q3b. If YES and your resource is related to an ANIMAL model, what types of models are provided?

    Under MTA with CHDI

    Q3c. If YES and your resource is related to a CELL model, what types of models are provided?

    Other - TgHD and control animal derived

    Q4a. Is this activity supported as:

    A collaboration

    Q4b. Do you deposit what you supply in any kind of central repository?

    Disease

    HD

    Species

    Minipigs

    Available to external user

    Yes

    Full phenotypic character

    Male fertility failure at 13 months, At two years: fragmentation of Htt in brain, and testes, neuroinflamation, decrease in total creatine in thalamus, At two years: fragmentation of Htt in brain, and testes, neuroinflamation, decrease in total creatine in thalamus, At four years: mitochondria impairment in heart and muscles, At five years: starting biohavioral and motoric problems based on three available pairs at this age

    Please indicate the phenotypes

    Four generations of minipigs with N-terminal part of human mutated (128Q,523AA)HTT expressed under the human promoter at chromosome 1 (1q24-q25) of porcine DNA and their wild type sibling.

    List of genotypes or other subtypes

    Q5b. Cognitive function, No of models

    Q5b. Cognitive function, Available to external users

    Q5b. Cognitive function, Full phenotypic characterisation

    Q5b. Cognitive function, Nature of phenotype

    Q5b. Motor function, No of models

    Q5b. Motor function, Available to external users

    Q5b. Motor function, Full phenotypic characterisation

    Q5b. Motor function, Nature of phenotype

    Q5b. Physiological function, no of models

    Q5b. Physiological function, Available to external users

    Q5b. Physiological function, Full phenotypic characterisation

    Q5b. Physiological function, Nature of phenotype

    Q5b. Other function (please specify), no of models

    Please specify other function

    Q5b. Other function (please specify), Available to external users

    Q5b. Other function (please specify), Full phenotypic characterisation

    Q5b. Other function (please specify), Nature of phenotype

    Q6. Please indicate if your resource is already linked into European or international consortia or networks?

    Q7a. Is maintenance of this resource dependent on continued funding?

    Yes

    Q7b. If yes, when does the current funding period end?

    2020

    Q7c. What is the expected lifespan of the resource (in years)?

    15-20

    Q7d. Are there other plans affecting future use that it may be useful to know?

Types: Experimental Models
Member States: Czech Republic
Diseases: N/A
Years: 2016
Database Categories: N/A
Database Tags: N/A

Export as PDF