Signals from the lysosome: a control centre for cellular clearance and energy metabolism
C Settembre, A Fraldi, DL Medina… - Nature reviews Molecular …, 2013 - nature.com
For a long time, lysosomes were considered merely to be cellular'incinerators' involved in
the degradation and recycling of cellular waste. However, now there is compelling evidence …
the degradation and recycling of cellular waste. However, now there is compelling evidence …
Brain disorders due to lysosomal dysfunction
Recent studies of autophagic and lysosomal pathways have significantly changed our
understanding of lysosomes; once thought to be simple degradative and recycling centers …
understanding of lysosomes; once thought to be simple degradative and recycling centers …
[PDF][PDF] Transcriptional activation of lysosomal exocytosis promotes cellular clearance
Lysosomes are cellular organelles primarily involved in degradation and recycling
processes. During lysosomal exocytosis, a Ca 2+-regulated process, lysosomes are docked …
processes. During lysosomal exocytosis, a Ca 2+-regulated process, lysosomes are docked …
A block of autophagy in lysosomal storage disorders
C Settembre, A Fraldi, L Jahreiss… - Human molecular …, 2008 - academic.oup.com
Most lysosomal storage disorders (LSDs) are caused by deficiencies of lysosomal
hydrolases. While LSDs were among the first inherited diseases for which the underlying …
hydrolases. While LSDs were among the first inherited diseases for which the underlying …
[HTML][HTML] Gene therapy for mucopolysaccharidoses: in vivo and ex vivo approaches
A Fraldi, M Serafini, NC Sorrentino, B Gentner… - Italian journal of …, 2018 - Springer
Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders caused by a
deficiency in lysosomal enzymes catalyzing the stepwise degradation of …
deficiency in lysosomal enzymes catalyzing the stepwise degradation of …
Binding of the 7SK snRNA turns the HEXIM1 protein into a P‐TEFb (CDK9/cyclin T) inhibitor
AA Michels, A Fraldi, Q Li, TE Adamson… - The EMBO …, 2004 - embopress.org
The positive transcription elongation factor b (P‐TEFb) plays a pivotal role in productive
elongation of nascent RNA molecules by RNA polymerase II. Core active P‐TEFb is …
elongation of nascent RNA molecules by RNA polymerase II. Core active P‐TEFb is …
Intracerebral Administration of Adeno-Associated Viral Vector Serotype rh.10 Carrying Human SGSH and SUMF1 cDNAs in Children with Mucopolysaccharidosis …
M Tardieu, M Zérah, B Husson… - Human gene …, 2014 - liebertpub.com
Mucopolysaccharidosis type IIIA is a severe degenerative disease caused by an autosomal
recessive defect of a gene encoding a lysosomal heparan-N-sulfamidase, the N …
recessive defect of a gene encoding a lysosomal heparan-N-sulfamidase, the N …
Lysosomal fusion and SNARE function are impaired by cholesterol accumulation in lysosomal storage disorders
A Fraldi, F Annunziata, A Lombardi, HJ Kaiser… - The EMBO …, 2010 - embopress.org
The function of lysosomes relies on the ability of the lysosomal membrane to fuse with
several target membranes in the cell. It is known that in lysosomal storage disorders (LSDs) …
several target membranes in the cell. It is known that in lysosomal storage disorders (LSDs) …
MAQ1 and 7SK RNA interact with CDK9/cyclin T complexes in a transcription-dependent manner
AA Michels, A Fraldi, V Labas, M Edwards… - … and cellular biology, 2003 - Am Soc Microbiol
Positive transcription elongation factor b (P-TEFb) comprises a cyclin (T1 or T2) and a
kinase, cyclin-dependent kinase 9 (CDK9), which phosphorylates the carboxyl-terminal …
kinase, cyclin-dependent kinase 9 (CDK9), which phosphorylates the carboxyl-terminal …
Lysosomal storage diseases as disorders of autophagy
The cellular turnover of proteins and organelles requires cooperation between the
autophagic and the lysosomal degradation pathways. A crucial step in this process is the …
autophagic and the lysosomal degradation pathways. A crucial step in this process is the …