Title : Upregulation of tripeptidyl-peptidase 1 by 3-hydroxy-(2,2)-dimethyl butyrate, a brain endogenous ligand of PPARĪ±: Implications for late-infantile Batten disease therapy.

Pub. Date : 2019 Jul

PMID : 30928643






6 Functional Relationships(s)
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1 Upregulation of tripeptidyl-peptidase 1 by 3-hydroxy-(2,2)-dimethyl butyrate, a brain endogenous ligand of PPARalpha: Implications for late-infantile Batten disease therapy. 3-hydroxy-2,2-dimethylbutyrate peroxisome proliferator activated receptor alpha Mus musculus
2 Moreover, by using primary astrocytes from wild type, PPARalpha-/- and PPARbeta-/- mice, we demonstrated that HDMB specifically required PPARalpha for inducing TPP1 expression. 3-hydroxy-2,2-dimethylbutyrate peroxisome proliferator activated receptor alpha Mus musculus
3 Moreover, by using primary astrocytes from wild type, PPARalpha-/- and PPARbeta-/- mice, we demonstrated that HDMB specifically required PPARalpha for inducing TPP1 expression. 3-hydroxy-2,2-dimethylbutyrate peroxisome proliferator activated receptor alpha Mus musculus
4 Moreover, by using primary astrocytes from wild type, PPARalpha-/- and PPARbeta-/- mice, we demonstrated that HDMB specifically required PPARalpha for inducing TPP1 expression. 3-hydroxy-2,2-dimethylbutyrate peroxisome proliferator activated receptor alpha Mus musculus
5 Finally, oral administration of HDMB to Cln2 heterozygous (Cln2+/-) mice led to a marked upregulation of TPP1 expression in the motor cortex and striatum in a PPARalpha-dependent fashion. 3-hydroxy-2,2-dimethylbutyrate peroxisome proliferator activated receptor alpha Mus musculus
6 Our study suggests that HDMB, a brain endogenous ligand of PPARalpha, might have therapeutic importance for LINCL treatment. 3-hydroxy-2,2-dimethylbutyrate peroxisome proliferator activated receptor alpha Mus musculus