In vitro and in vivo plasmalogen replacement evaluations in rhizomelic chrondrodysplasia punctata and Pelizaeus-Merzbacher disease using PPI-1011, an ether lipid plasmalogen precursor

Lipids in Health and Disease

Volumn 10, Issue , 2011, Pages

  Wood, Paul L ^a   Khan, M Amin ^b   Smith, Tara ^b   Ehrmantraut, Greg ^b   Jin, Wei ^b   Cui, Wei ^c   Braverman, Nancy E ^c   Goodenowe, Dayan B ^b  

^a Lincoln Memorial University   (United States)

^b Phenomenome Discoveries Inc   (Canada)

^c MONTREAL CHILDREN S HOSPITAL   (Canada)

Author keywords

DHA; lymphocytes; Pelizaeus Merzbacher disease; peroxisomal disorders; Pex7 mouse; plasmalogen precursor; PPI 1011; Rhizomelic chrondrodysplasia punctata type 1; Rhizomelic chrondrodysplasia punctata type 2

Indexed keywords

ETHANOLAMINE; ETHER LIPID; PLASMALOGEN; PPI 1011; PPI 1038; UNCLASSIFIED DRUG; CELL RECEPTOR; DIACYLGLYCEROL; PEROXISOMAL TARGETING SIGNAL 2 RECEPTOR; PHOSPHATIDAL ETHANOLAMINES;

ACYLATION AND DEACYLATION; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CHONDRODYSPLASIA PUNCTATA; CONCENTRATION RESPONSE; CONTROLLED STUDY; ENDOPLASMIC RETICULUM; GENE MUTATION; IN VITRO STUDY; IN VIVO STUDY; LYMPHOCYTE; MISSENSE MUTATION; MOUSE; NONHUMAN; PELIZAEUS MERZBACHER DISEASE; PEROXISOME; PROTEIN FOLDING; TREATMENT DURATION; ANIMAL; BIOSYNTHESIS; CELL CULTURE; DRUG EFFECT; EYE; GENETICS; HUMAN; KIDNEY; METABOLISM; MOUSE MUTANT; NEOCORTEX; TISSUE DISTRIBUTION;

ANIMALS; CELLS, CULTURED; CHONDRODYSPLASIA PUNCTATA, RHIZOMELIC; DIGLYCERIDES; EYE; HUMANS; KIDNEY; LYMPHOCYTES; MICE; MICE, KNOCKOUT; NEOCORTEX; PELIZAEUS-MERZBACHER DISEASE; PLASMALOGENS; RECEPTORS, CYTOPLASMIC AND NUCLEAR; TISSUE DISTRIBUTION;

EID: 80054118480     PISSN: None     EISSN: 1476511X     Source Type: Journal    
DOI: 10.1186/1476-511X-10-182     Document Type: Article

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