Center for Inherited Disorder Services Offered of Energy Metabolism (CIDEM)

______Wearn Bldg., Room 649

Human Clinical Samples University Hospitals Case Medical Center 11100 Euclid Avenue, Cleveland OH 44106 CIDEM

Quantitative Analysis Analysis (including and Tel: 216-844-1286 monolysocardiolipin molecular species) Fax:216-844-8005 Center for Inherited  Isolated Skeletal Muscle Mitochondria [email protected] Disorders of Energy Cardiolipin and Monolysocardiolipin Analysis www.case.edu/med/CIDEM/  Skeletal Muscle Metabolism  Skin Fibroblasts Tax ID: 34-1720429 CLIA: 36D0680824 ww.case.edu/med/CIDEM Available Fall 2012! CLIA: 36D0925804 Quantitative Analysis NPI: 1578706438 Phospholipid Analysis (including cardiolipin and monolysocardiolipin molecular species) Quantitative  Skeletal Muscle (frozen/fresh) Isolated Human Skeletal Muscle Mitochondria  Fibroblasts Reference Intervals: Box = Mean ±SD; Whiskers = Range  Blood Phospholipid Analysis 300 1.4 ______n 250 1.2 by HPLC-MS 1 protei

200 Team Members 0.8

mito 150 Charles Hoppel, MD 0.6 Juhnwan Kim, PhD 100 0.4 Paul Minkler 50 nmol/mg 0.2 0 0 CL PE PC PI PG

Quality Control Results for Cardiolipin Center for Mitochondrial Diseases Rat Skeletal Muscle Mitochondria, N = 48, 11% RSD Case Western Reserve University School of Medicine Department of Pharmacology 10900 Euclid Avenue Cleveland, Ohio 44106-4981 Center for Mitochondrial Diseases Among the nation’s leading academic medical cen- 70 Case Western Reserve University School of Medicine 60 ters, University Hospitals Case Medical Center is the Department of Pharmacology 50 primary affiliate of Case Western Reserve University 10900 Euclid Avenue 40 School of Medicine, a nationally recognized leader in 30 Cleveland, Ohio 44106-4981 20 medical research and education. 10

0

2011/ 07 2012/ 06

Human Skeletal Muscle Mitochondria Human Skin Fibroblasts Human Skeletal Muscle Phospholipid HPLC-MS Chromatogram and Spectra Phospholipid HPLC-MS Chromatogram and Spectra Phospholipid HPLC-MS Chromatogram and Spectra

747.5 766.7 775.7 766.7 766.7 747.6

885.8 788.6 885.8 885.8 599.4 836.5

1452.3 1448.1 1185.7 1163.4 1448.0 786.7

804.7 747.7 1185.7 802.9 802.8

O O OH Symbol Key: O 540.3 O 747.6 O PE - Oxidized monohydroperoxides PG - PI - Cardiolipin O OO H OO H O LPI - lysophosphatidylinositol Reversed- O PS - O Phase Ion-Pair dihydroperoxides CL - cardiolipin MLCL - monolysocardiolipin HPLC-MS/MS O O OH O PC - O O OH SM - O bis monohydroperoxides LPC - lysophosphatidylcholine