Ontology highlight
ABSTRACT:
SUBMITTER: Collard R
PROVIDER: S-EPMC5829486 | biostudies-literature | 2018 Mar
REPOSITORIES: biostudies-literature
Collard Renata R Majtan Tomas T Park Insun I Kraus Jan P JP
Molecular and cellular biology 20180227 6
Propionic acidemia is caused by a deficiency of the enzyme propionyl coenzyme A carboxylase (PCC) located in the mitochondrial matrix. Cell-penetrating peptides, including transactivator of transcription (TAT), offer a potential to deliver a cargo into the mitochondrion. Here, we investigated the delivery of an α<sub>6</sub>β<sub>6</sub> PCC enzyme into mitochondria using the HIV TAT peptide at several levels: into isolated mitochondria, in patient fibroblast cells, and in a mouse model. Results ...[more]