Cln5 cDNA ORF clone, Mus musculus(house mouse)

Loss of
Savchenko E, Singh Y, Konttinen H, Lejavova K, Mediavilla Santos L, Grubman A, K?rkk?inen V, Keksa-Goldsteine V, Naumenko N, Tavi P, White AR, Malm T, Koistinaho J, Kanninen KM
Disease models & mechanisms10(9)1089-1100(2017 Sep)

Retinal Degeneration In A Mouse Model Of CLN5 Disease Is Associated With Compromised Autophagy.
Leinonen H, Keksa-Goldsteine V, Ragauskas S, Kohlmann P, Singh Y, Savchenko E, Puranen J, Malm T, Kalesnykas G, Koistinaho J, Tanila H, Kanninen KM
Scientific reports7(1)1597(2017 May)

Neuronal ceroid lipofuscinosis genes, CLN2, CLN3 and CLN5 are spatially and temporally co-expressed in a developing mouse brain.
Fabritius AL, Vesa J, Minye HM, Nakano I, Kornblum H, Peltonen L
Experimental and molecular pathology97(3)484-91(2014 Dec)

Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice.
Blom T, Schmiedt ML, Wong AM, Kytt?l? A, Soronen J, Jauhiainen M, Tyynel? J, Cooper JD, Jalanko A
Disease models & mechanisms6(2)342-57(2013 Mar)

Rssults suggested that CLN2, CLN3 and CLN5 genes may play an important role in early embryonal neurogenesis.
Title: Neuronal ceroid lipofuscinosis genes, CLN2, CLN3 and CLN5 are spatially and temporally co-expressed in a developing mouse brain.

The simultaneous loss of both Cln1 and Cln5 genes might enhance the typical pathological phenotypes of these mice by disrupting or downregulating shared or convergent pathogenic pathways.
Title: Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice.

In Cln5-deficient mice, early and significant microglial activation is documented that is already evident at 3 months of age; it increases gradually with age and differs between neurons and glia, with the highest expression in microglia.
Title: Cln5-deficiency in mice leads to microglial activation, defective myelination and changes in lipid metabolism.

Study found that CLN5 interacts with several other NCL proteins namely, CLN1/PPT1, CLN2/TPP1, CLN3, CLN6 and CLN8.
Title: Novel interactions of CLN5 support molecular networking between Neuronal Ceroid Lipofuscinosis proteins.

These data provide unexpected evidence for a distinctive sequence of neuron loss in the thalamocortical system of Cln5-/- mice, diametrically opposed to that seen in other forms of NCL.
Title: Progressive thalamocortical neuron loss in Cln5 deficient mice: Distinct effects in Finnish variant late infantile NCL.