Primary systemic carnitine deficiency (SCD; OMIM 212140) is an autosomal recessive disorder
characterized by progressive cardiomyopathy, skeletal myopathy, hypoglycaemia and …

Diabetic nephropathy is a major microvascular complication in long-standing diabetic patients
who eventually undergo renal dialysis or transplantation. To prevent development of this …

Asparaginyl endopeptidase (AEP)/legumain, an asparagine-specific cysteine proteinase in
animals, is an ortholog of plant vacuolar processing enzyme (VPE), which processes the …

Transport of l‐[ 3 H]carnitine and acetyl‐l‐[ 3 H]carnitine at the blood–brain barrier (BBB)
was examined by using in vivo and in vitro models. In vivo brain uptake of acetyl‐l‐[ 3 H]…

OCTN2 is an Na + -dependent transporter for carnitine, which is essential for fatty acid
metabolism, and its functional defect leads to fatal systemic carnitine deficiency (SCD). It also …

Selectins recognize ligands containing carbohydrate chains such as sialyl Lewis x (sLe x )
that are mainly presented at the terminus of N-acetyl lactosamine repeats on core 2 O-glycans…

Asparaginyl endopeptidase (AEP or legumain) is a lysosomal cysteine protease that cleaves
protein substrates on the C-terminal side of asparagine. AEP plays a pivotal role in the …

β4 Galactosylation of glycoproteins plays important roles in protein conformation, stability,
transport, and clearance from the circulation. Recent studies have revealed that aberrant …

Advanced glycation end products (AGEs) and the receptor for AGE (RAGE) interactions
have been implicated in the development of diabetic vascular complications, which cause …

We kinetically analyzed the disposition of l-carnitine of juvenile visceral steatosis (JVS) mice
compared with that of normal mice to elucidate the mechanism of the systemic l-carnitine …