Animal models of human genetic diseases: do they need to be faithful to be useful? (Q37872654)

24 August 2017

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title

Animal models of human genetic diseases: do they need to be faithful to be useful? (English)

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24 August 2017

Jean-Louis Guénet

1

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24 August 2017

publication date

6 May 2011

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Molecular Genetics and Genomics

24 August 2017

published in

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24 August 2017

volume

286

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24 August 2017

issue

1

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24 August 2017

page(s)

1-20

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Pancreatic islet expression profiling in diabetes-prone C57BLKS/J mice reveals transcriptional differences contributed by DBA loci, including Plagl1 and Nnt

24 August 2017

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Experimental hemochromatosis due to MHC class I HFE deficiency: immune status and iron metabolism

21 January 2018

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Gene inactivation and its implications for annotation in the era of personal genomics

21 January 2018

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The modifier of Min 2 (Mom2) locus: embryonic lethality of a mutation in the Atp5a1 gene suggests a novel mechanism of polyp suppression.

21 January 2018

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https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Targeted disruption of the mouse factor VIII gene produces a model of haemophilia A

21 January 2018

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https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Transgenic mouse model of the mild dominant form of osteogenesis imperfecta

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Ferrochelatase structural mutant (Fechm1Pas) in the house mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

X chromosome-linked muscular dystrophy (mdx) in the mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

The frissonnant mutant mouse, a model of dopamino-sensitive, inherited motor syndrome

21 January 2018

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https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Genetic Modification of the Phenotypes Produced by Amyloid Precursor Protein Overexpression in Transgenic Mice

21 January 2018

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https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Duchenne muscular dystrophy models show their age.

21 January 2018

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https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Angelman Syndrome, a Genomic Imprinting Disorder of the Brain

21 January 2018

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Genetic Defects in Muscular Dystrophy

21 January 2018

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A Human-Specific Deletion in Mouse Cmah Increases Disease Severity in the mdx Model of Duchenne Muscular Dystrophy

21 January 2018

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https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Recovery of induced mutations for X chromosome-linked muscular dystrophy in mice

21 January 2018

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https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

A mutation in the human leptin receptor gene causes obesity and pituitary dysfunction

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

The influence of genetic background on the expression of the obese (ob) gene in the mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

A candidate gene for human neurodegenerative disorders: a rat PKC gamma mutation causes a Parkinsonian syndrome.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Mouse alleles: if you've seen one, you haven't seen them all.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Ultrafine mapping of SNPs from mouse strains C57BL/6J, DBA/2J, and C57BLKS/J for loci contributing to diabetes and atherosclerosis susceptibility

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Genetic animal models of Parkinson's disease

21 January 2018

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https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Utrophin-dystrophin-deficient mice as a model for Duchenne muscular dystrophy

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Genetic identification of Mom-1, a major modifier locus affecting Min-induced intestinal neoplasia in the mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

The mousetrap: what we can learn when the mouse model does not mimic the human disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Two new mutants, ‘trembler’ and ‘reeler’, with neurological actions in the house mouse (Mus musculus L.)

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

FOXP2 as a molecular window into speech and language

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Generation of mice with a conditional Foxp2 null allele

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Animal models of Alzheimer's disease and frontotemporal dementia

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Localized gene action controlling intestinal neoplasia in mice

21 January 2018

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https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Patterns and mechanisms of genome organization in the mouse

21 January 2018

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https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Wild mice: an ever-increasing contribution to a popular mammalian model.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Cystic fibrosis mouse models

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Transgenic rescue implicates beta2-microglobulin as a diabetes susceptibility gene in nonobese diabetic (NOD) mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Tumour predisposition in mice heterozygous for a targeted mutation in Nf1

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

The human gene for alkaptonuria (AKU) maps to chromosome 3q.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Three new allelic mouse mutations that cause skeletal overgrowth involve the natriuretic peptide receptor C gene (Npr3).

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Mutation of the Angelman ubiquitin ligase in mice causes increased cytoplasmic p53 and deficits of contextual learning and long-term potentiation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Lesch-Nyhan disease: from mechanism to model and back again

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Ectopic expression of the agouti gene in transgenic mice causes obesity, features of type II diabetes, and yellow fur

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

A potential animal model for Lesch-Nyhan syndrome through introduction of HPRT mutations into mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

A mutation in the gene encoding mitochondrial Mg²+ channel MRS2 results in demyelination in the rat.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Identification of Mom7, a novel modifier of Apc(Min/+) on mouse chromosome 18.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

A transgenic model for listeriosis: role of internalin in crossing the intestinal barrier

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Sub-physiological sarcoglycan expression contributes to compensatory muscle protection in mdx mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

A new mouse model for marfan syndrome presents phenotypic variability associated with the genetic background and overall levels of Fbn1 expression

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

The secretory phospholipase A2 gene is a candidate for the Mom1 locus, a major modifier of ApcMin-induced intestinal neoplasia

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

FOXP2 in focus: what can genes tell us about speech and language?

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

A missense mutation in Tbce causes progressive motor neuronopathy in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

The impact of genetic research on our understanding of Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Animal models of brain dysfunction in phenylketonuria

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Griscelli syndrome type 2: a rare and lethal disorder.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Griscelli syndrome types 1 and 2.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Animal models of inherited neuropathies

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

aku, a mutation of the mouse homologous to human alkaptonuria, maps to chromosome 16

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

A dominant mutation that predisposes to multiple intestinal neoplasia in the mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

The Min (multiple intestinal neoplasia) mutation: its effect on gut epithelial cell differentiation and interaction with a modifier system

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Loss of Rab25 promotes the development of intestinal neoplasia in mice and is associated with human colorectal adenocarcinomas

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Telomere lengths and telomerase activity in dog tissues: a potential model system to study human telomere and telomerase biology

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Measles virus infection in a transgenic model: virus-induced immunosuppression and central nervous system disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Griscelli disease maps to chromosome 15q21 and is associated with mutations in the myosin-Va gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Correction of sickle cell disease in transgenic mouse models by gene therapy

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

A new mouse model for the trisomy of the Abcg1-U2af1 region reveals the complexity of the combinatorial genetic code of down syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Two hypomorphic alleles of mouse Ass1 as a new animal model of citrullinemia type I and other hyperammonemic syndromes

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Evidence of a large-scale functional organization of mammalian chromosomes

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Dramatically different phenotypes in mouse models of human Tay-Sachs and Sandhoff diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Differential expression of dystrophin isoforms in strains of mdx mice with different mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Transgenic mice expressing a human poliovirus receptor: a new model for poliomyelitis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Understanding the basis for Down syndrome phenotypes

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Microphthalmia, parkinsonism, and enhanced nociception in Pitx3 ( 416insG ) mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Modulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Short telomeres and stem cell exhaustion model Duchenne muscular dystrophy in mdx/mTR mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Identification of the modifier of Min 2 (Mom2) locus, a new mutation that influences Apc-induced intestinal neoplasia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Multiple intestinal neoplasia caused by a mutation in the murine homolog of the APC gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Behavioral effects of a deletion in Kcnn2, the gene encoding the SK2 subunit of small-conductance Ca2+-activated K+ channels

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Erythropoietic protoporphyria in the house mouse. A recessive inherited ferrochelatase deficiency with anemia, photosensitivity, and liver disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

'Too much good news' - are Alzheimer mouse models trying to tell us how to prevent, not cure, Alzheimer's disease?

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Positional cloning of the mouse obese gene and its human homologue

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

The Collaborative Cross, a community resource for the genetic analysis of complex traits

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Amelioration of the dystrophic phenotype of mdx mice using a truncated utrophin transgene

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00438-011-0627-Y

Animal models of Alzheimer's disease: therapeutic implications.

21 January 2018

1 reference

12 December 2020

Mutation of TBCE causes hypoparathyroidism-retardation-dysmorphism and autosomal recessive Kenny-Caffey syndrome

1 reference

12 December 2020

Mouse models of human phenylketonuria

1 reference

12 December 2020

Secretory phospholipase Pla2g2a confers resistance to intestinal tumorigenesis

1 reference

12 December 2020

Why isn't a mouse more like a man?

1 reference

12 December 2020

Alopecia and male infertility in oligotriche mutant mice are caused by a deletion on distal chromosome 9

1 reference

12 December 2020

A deletion in the gene encoding sphingomyelin phosphodiesterase 3 (Smpd3) results in osteogenesis and dentinogenesis imperfecta in the mouse

1 reference

12 December 2020

Targeted inactivation of the coagulation factor IX gene causes hemophilia B in mice.

1 reference

12 December 2020

Limitations of animal models of Parkinson's disease

1 reference

12 December 2020

A candidate mouse model for Prader-Willi syndrome which shows an absence of Snrpn expression

1 reference

12 December 2020

Obese, a new mutation in the house mouse

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12 December 2020

Transgenic knockout mice with exclusively human sickle hemoglobin and sickle cell disease

1 reference

12 December 2020

Do non-linked genes really reassort at random?

1 reference

12 December 2020

Dnmt1N/+ reduces the net growth rate and multiplicity of intestinal adenomas in C57BL/6-multiple intestinal neoplasia (Min)/+ mice independently of p53 but demonstrates strong synergy with the modifier of Min 1(AKR) resistance allele

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12 December 2020

Telomere length in normal and neoplastic canine tissues

1 reference

12 December 2020

Diabetes and Obesity: Thrifty Mutants?

1 reference

12 December 2020

10.1007/S00438-011-0627-Y

Identifiers

DOI

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24 August 2017

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