STOCK Hsd17b4^tm2Baes/Orl

Status	Available to order
EMMA ID	EM:13784
Citation information	RRID:IMSR_EM:13784 Research Resource Identifiers (RRID) are persistent unique ID numbers assigned to help researchers cite key resources (e.g. antibodies, model organisms and software projects) in the biomedical literature to improve transparency and reproducibility in research. See https://www.rrids.org/ for more information.
International strain name	STOCK Hsd17b4^tm2Baes/Orl
Alternative name	HSD17B4 floxed
Strain type	Targeted Mutant Strains : Conditional mutation
Allele/Transgene symbol	Hsd17b4^tm2Baes
Gene/Transgene symbol	Hsd17b4

Information from provider

Provider	Myriam Baes
Provider affiliation	Pharmaceutical and pharmacological sciences, KU Leuven
Genetic information	Two loxP sites are integrated in introns surrounding exon 8 of Hsd17b4
Phenotypic information	Homozygous: Homozygous floxed Hsd17b4 mice are normal throughout their lifetime Heterozygous: Heterozygous floxed Hsd17b4 mice are normal throughout their lifetime
Breeding history	Inbred for at least 8 generations in the Swiss background
References	Autonomous Purkinje cell axonal dystrophy causes ataxia in peroxisomal multifunctional protein-2 deficiency.;De Munter Stephanie, Bamps Dorien, Malheiro Ana Rita, Kumar Baboota Ritesh, Brites Pedro, Baes Myriam, ;2018;Brain pathology (Zurich, Switzerland);28;631-643; 29341299 Neuronal Dysfunction and Behavioral Abnormalities Are Evoked by Neural Cells and Aggravated by Inflammatory Microglia in Peroxisomal β-Oxidation Deficiency.;Beckers Lien, Stroobants Stijn, D'Hooge Rudi, Baes Myriam, ;2018;Frontiers in cellular neuroscience;12;136; 29892213 Microglia lacking a peroxisomal β-oxidation enzyme chronically alter their inflammatory profile without evoking neuronal and behavioral deficits.;Beckers Lien, Geric Ivana, Stroobants Stijn, Beel Sander, Van Damme Philip, D'Hooge Rudi, Baes Myriam, ;2019;Journal of neuroinflammation;16;61; 30866963 Peroxisomal multifunctional protein-2 deficiency causes neuroinflammation and degeneration of Purkinje cells independent of very long chain fatty acid accumulation.;Verheijden Simon, Bottelbergs Astrid, Krysko Olga, Krysko Dmitri V, Beckers Lien, De Munter Stephanie, Van Veldhoven Paul P, Wyns Sabine, Kulik Wim, Nave Klaus-Armin, Ramer Matt S, Carmeliet Peter, Kassmann Celia M, Baes Myriam, ;2013;Neurobiology of disease;58;258-69; 23777740
Homozygous fertile	yes
Homozygous viable	yes
Homozygous matings required	no
Immunocompromised	no

Information from EMMA

Archiving centre	CNRS-TAAM – Typing and Archiving of Animal Models, Orléans, France
Animals used for archiving	homozygous males

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

Perrault syndrome / Orphanet_2855
Bifunctional enzyme deficiency / Orphanet_300

IMPC phenotypes (gene matching)

abnormal spleen morphology / IMPC
abnormal sternum morphology / IMPC
small uterus / IMPC
enlarged heart / IMPC
abnormal iris morphology / IMPC
limb grasping / IMPC
enlarged adrenal glands / IMPC
decreased circulating aspartate transaminase level / IMPC
abnormal gait / IMPC
preweaning lethality, incomplete penetrance / IMPC
cataract / IMPC
abnormal auditory brainstem response / IMPC
enlarged urinary bladder / IMPC
aspermia / IMPC
abnormal eye morphology / IMPC
irregularly shaped pupil / IMPC
hypoplasia / IMPC
cataract / IMPC
abnormal tooth morphology / IMPC
follicular atresia / IMPC
increased grip strength / IMPC
abnormal embryo size / IMPC
dysplasia / IMPC
abnormal seminal vesicle morphology / IMPC

Literature references

Autonomous Purkinje cell axonal dystrophy causes ataxia in peroxisomal multifunctional protein-2 deficiency.;De Munter Stephanie, Bamps Dorien, Malheiro Ana Rita, Kumar Baboota Ritesh, Brites Pedro, Baes Myriam, ;2018;Brain pathology (Zurich, Switzerland);28;631-643; 29341299
Neuronal Dysfunction and Behavioral Abnormalities Are Evoked by Neural Cells and Aggravated by Inflammatory Microglia in Peroxisomal β-Oxidation Deficiency.;Beckers Lien, Stroobants Stijn, D'Hooge Rudi, Baes Myriam, ;2018;Frontiers in cellular neuroscience;12;136; 29892213
Microglia lacking a peroxisomal β-oxidation enzyme chronically alter their inflammatory profile without evoking neuronal and behavioral deficits.;Beckers Lien, Geric Ivana, Stroobants Stijn, Beel Sander, Van Damme Philip, D'Hooge Rudi, Baes Myriam, ;2019;Journal of neuroinflammation;16;61; 30866963
Peroxisomal multifunctional protein-2 deficiency causes neuroinflammation and degeneration of Purkinje cells independent of very long chain fatty acid accumulation.;Verheijden Simon, Bottelbergs Astrid, Krysko Olga, Krysko Dmitri V, Beckers Lien, De Munter Stephanie, Van Veldhoven Paul P, Wyns Sabine, Kulik Wim, Nave Klaus-Armin, Ramer Matt S, Carmeliet Peter, Kassmann Celia M, Baes Myriam, ;2013;Neurobiology of disease;58;258-69; 23777740

Information on how we integrate external resources can be found here

Order

Availabilities

Requesting frozen sperm or embryos is generally advisable wherever possible, in order to minimise the shipment of live mice.

Frozen sperm. Delivered in 4 weeks (after paperwork in place). €1740^*
Rederivation of mice from frozen stock, delivery time available upon request . €3880^*

Due to the dynamic nature of our processes strain availability may change at short notice. The local repository manager will advise you in these circumstances.

^* In addition users have to cover all the shipping costs (including the cost for returning dry-shippers, where applicable).

More details on pricing and delivery times

Practical information

Genotyping protocol

Example health report
(Current health report will be provided later)

Material Transfer Agreement (MTA)
Distribution of this strain is subject to a provider MTA. Both signing of the MTA and submission of the online EMMA Mutant Request Form are required before material can be shipped.

EMMA conditions
Legally binding conditions for the transfer

Right strain for your research?

The information provided on this page is, to the best of EMMA’s knowledge, based on data supplied by the original provider. End users are responsible for reviewing these details and for validating the strain and its suitability for their experimental use.
Not found what you were looking for? Search here for other strains available from EMMA.

STOCK Hsd17b4tm2Baes/Orl