Any feedback?
Please rate this page
(literature.php)
(0/150)

BRENDA support

Literature summary for 3.4.24.B18 extracted from

  • Murru, S.; Hess, S.; Barth, E.; Almajan, E.R.; Schatton, D.; Hermans, S.; Brodesser, S.; Langer, T.; Kloppenburg, P.; Rugarli, E.I.
    Astrocyte-specific deletion of the mitochondrial m-AAA protease reveals glial contribution to neurodegeneration (2019), Glia, 67, 1526-1541 .
    View publication on PubMedView publication on EuropePMC
Search for more literature for 3.4.24.B18

Organism

Organism UniProt Comment Textmining
Mus musculus Q920A7 and Q8JZQ2 Q920A7 i.e. subunit Afg3l1, Q8JZQ2 i.e. subunit Afg3l2
-

Source Tissue

Source Tissue Comment Organism Textmining
astrocyte
-
 Mus musculus
-
Bergmanns glia radial astrocytes of the cerebellum Mus musculus
-

Synonyms

Synonyms Comment Organism
Afg3l1
-
 Mus musculus
AFG3L2
-
 Mus musculus

General Information

General Information Comment Organism
physiological function astrocyte-specific deletion of Afg3l2 in the mouse leads to late-onset motor impairment and to degeneration of Bergmann glia, which display aberrant morphology, altered expression of the glutamate transporter EAAT2, and a reactive inflammatory signature. The neurological and glial phenotypes are drastically exacerbated when astrocytes lack both subunits Afg31l and Afg3l2. Mitochondrial stress responses and necroptotic markers are induced in the cerebellum. In both mouse models, targeted Bergmann glia show a fragmented mitochondrial network and loss of mitochondrial cristae, but no signs of respiratory dysfunction. Astrocyte-specific deficiency of Afg3l1 and Afg3l2 triggers secondary morphological degeneration and electrophysiological changes in Purkinje cells Mus musculus