04.Basillica di Santa Maria degli Angeli, Rome, July 2010, after licensing meeting with Sigma-tau
10.Fig. 1.3: The process of transformation of meal worm (Tenebrio molitor) from larva → pupa → adult. Vitamin BT (L-carnitine) was found to be an essential nutrient for transformation from larva to pupa. (From Sankyo Remake Co., Ltd. Home page, with permission, http://www.sankyoremake.com/syokug_control_s.html)
11.Fig. 2.5: Carnitine transport by Chinese hamster ovary (CHO) cells expressing normal and mutant carnitine transporters identified from symptomatic patients and asymptomatic mothers. Panel a: Data for individual missense mutations of symptomatic patients and asymptomatic mothers. Points are averages ± SE of six samples. Panel b: The average for mutations identified in symptomatic patients and asymptomatic mothers. Note the logarithmic scale in panel b. In both groups, carnitine transport was significantly (P < 0.01 using ANOVA) reduced as compared to wild-type OCTN2, but there was no significant difference between the groups of missense mutations identified in symptomatic patients versus asymptomatic mothers using ANOVA. ANOVA analysis of variance, SE standard error. (From Ref. [84])
12.Table 3.8: Acronyms, names, subcellular localization, acyl-chain specificity, and malonyl-CoA sensitivity of various carnitine acyltransferases
13.Table 4.6: Organ distribution of activities of four biosynthesis enzymes of carnitine
14.Table 5.2: Common features of fatty acid oxidation disorders
15.Table 6.7: Pharmaceutical products of DL-carnitine sold in Japan in the past
16.Table 7.1: ecNOS and HO-1 gene (A) and protein expression (B) in human umbilical endothelial cells incubated with H2O2 alone and with H2O2 and L-carnitine, or acetyl-L-carnitine, or propionyl-L-carnitine
17.Table 8.1: Carnitine content and distribution of various tissues and organs of the human body
18.Table 9.2: Total trimethylamine (TMA) contents in various fishes and urinary excretion rate
19.Table 10.7: Classification of mitochondrial abnormalities by functions
20.Table 11.10: Case reports of carnitine deficiency caused by Specific milks such as milk for allergies in Japan
21.Table 12.14: Description of carnitine administration for hyperammonemia due to valproic acid poisoning in the Washington Manual
22.Table 13.2: Case reports of carnitine deficiency caused by pivoxil-containing antibiotics in Japan
23.Table 14.13: Proportion of kidney transplantation in patients with ESRD in each country
24.Table 15.14: Histological data in patients treated with placebo plus diet or L-carnitine plus diet before and after 24 weeks of treatment
25.Table 16.10: Individual metabolites of long-chain acylcarnitine means and comparisons between HFpEF, HFrEF, and No-HF controls
26.Fig. 17.5: Effects of L-carnitine on survival. Non-Tg and Tg littermates were treated as in Fig. 17.3. Proportions of survival were plotted with Kaplan-Meier curves, and the difference between Tg and Tg animals treated with L-carnitine was tested by the log-rank test (P = 0.0001). (From Ref. [15])
27.Table 18.3: Reference values for carnitine measurement by enzyme cycling method (blood carnitine 2 fractions test)
28.Table 19.2: Status of approvals of off-label drugs and unapproved drugs covered in “Formulation of Priority List of Off-Label Drugs and Unapproved Drugs in the Field of Inherited Metabolic Diseases” in Japan (as of the end of August 2022)
