Tg(AR*100Q)C25Als
Transgene Detail

Summary

• Symbol: Tg(AR*100Q)C25Als
• Name: transgene insertion C25, Albert La Spada
• MGI ID: MGI:5000265
• Synonyms: AR100, AR YAC CAG100
• Transgene: Tg(AR*100Q)C25Als Location: unknown
• Alliance: Tg(AR*100Q)C25Als page

Transgene origin

• Germline Transmission: Earliest citation of germline transmission: J:89772
• Parent Cell Line: R1 (ES Cell)
• Strain of Origin: (129X1/SvJ x 129S1/Sv)F1-Kitl⁺

Transgene description

• Transgene Type: Transgenic (Humanized sequence, Inserted expressed sequence)
• Mutation: Insertion
• Tg(AR*100Q)C25Als expresses 1 gene
  Transgene expresses:
  

  Organism

  Expressed Gene

  Homolog in Mouse

  Note

  human

  AR (367)

  Ar	(MGI:88064)

• Mutation details: The transgene contains a human YAC in which the CAG repeat contains 100 repeats (100Q). Lines C25 (high expressor) and C32 (low expressor) were generated. (J:89772)

Phenotypes

Key:

hm	homozygous	ht	heterozygous	tg	involves transgenes	√	phenotype observed
cn	conditional genotype	cx	complex: > 1 genome feature	ot	other: hemizygous, indeterminate,...	N	normal phenotype

Genotype/
Background:

	Allelic Composition	Genetic Background	Cell Line(s)
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tg1  Disease Model	Tg(AR*100Q)C25Als/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6J

Phenotypes:

Affected Systems	tg1
show or hide all annotated terms
behavior/neurological	√
ataxia	√
impaired coordination	√
abnormal gait	√
short stride length	√
hindlimb paralysis	√
growth/size/body	√
decreased body size	√
decreased body weight	√
postnatal growth retardation	√
liver/biliary system	√
abnormal liver morphology	√
mortality/aging	√
premature death	√
muscle	√
abnormal muscle morphology	√
skeletal muscle atrophy	√
muscle weakness	√
nervous system	√
motor neuron degeneration	√
skeleton	√
kyphosis	√

View phenotypes and curated references for all genotypes (concatenated display).

Disease models

Key:

√

disease model

expected model not found

Models:

Human Diseases	tg1
Kennedy's disease IDs Kennedy's disease       DOID:0060161 MESH:D055534 NCI:C85233 OMIM:313200 UMLS_CUI:C1839259 Close	√

Expression

In Structures Affected by this Mutation:

5 anatomical structure(s)

Find Mice (IMSR)

• Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
• Carrying this Mutation: Mouse Strains: 0 strains available Cell Lines: 0 lines available

References

• Original: J:89772 Sopher BL, et al., Androgen receptor YAC transgenic mice recapitulate SBMA motor neuronopathy and implicate VEGF164 in the motor neuron degeneration. Neuron. 2004 Mar 4;41(5):687-99
• All: 1 reference(s)