Mastocytosis, a rare blood disorder characterized by the proliferation of clonal abnormal mast cells, has variegated clinical spectrum and diagnosis is often difficult delayed. Recently we proposed cathepsin inhibitor cystatin D-R26 as salivary candidate biomarker systemic mastocytosis (SM). Its C26 variant able to form multiprotein complexes (mPCs) since protein–protein interactions (PPIs) are...

An investigation of the MeOH extract from the roots of Peritassa campestris (Hippocrateaceae) afforded two isomeric seco-A-ring quinonemethide triterpenoids, campestrine-I (1) and -II (2). This appears to be the first report of a C26,-type triterpene carbon skeleton from the Celastraceae or Hippocrateaceae families. The structures were elucidated on the basis of spectral data, particularly HMQC...

The proton pumping H+-ATPase, Pma1, is one of the most abundant integral membrane proteins of the yeast plasma membrane. Pma1 activity controls the intracellular pH and maintains the electrochemical gradient across the plasma membrane, two essential cellular functions. The maintenance of the proton gradient, on the other hand, also requires a specialized lipid composition of this membrane. The ...

Purpose: Mutational activation of the KRAS oncogene and overexpression of cyclooxygenase-2 (COX-2) contribute to colorectal carcinoma (CRC) development, but the relationship between these two events is unclear. This study was designed to clarify that relationship and to assess the contribution of KRAS-dependent COX-2 to the seeding of CRC cells in the liver and to their outgrowth as liver metas...

Protein hypercatabolism significantly contributes to the onset and progression of muscle wasting in cancer cachexia. In this regard, a major role is played by the ATP-ubiquitin-proteasome-dependent pathway and by autophagy. However, little is known about the relevance of the Ca2+-dependent proteolytic system. Since previous results suggested that this pathway is activated in the skeletal muscle...

Chain elongation and 2-hydroxylation pathways, which are specific for very long chain fatty acids ( >C&), have been found in the yeast, Candida utitis. The chain elongation system acts specifically on fatty acids of chain length C2,, to CZ4; there is no detectable elongation of Cl* and only trace . . . actrvrty with C&,. The product of the elongation enzyme or enzymes is C& when Co, C&, or C24 ...

The inherited peroxisomal disorder X-linked adrenoleukodystrophy (X-ALD), associated with neurodegeneration and inflammatory cerebral demyelination, is caused by mutations in the ABCD1 gene encoding the peroxisomal ATP-binding cassette (ABC) transporter ABCD1 (ALDP). ABCD1 transports CoA-esters of very long-chain fatty acids (VLCFA) into peroxisomes for degradation by β-oxidation; thus, ABCD1 d...

Cultured cell lines LL, B1 6, C26, and C38 establ ished from mouse solid tumors of Lewis lung carcinoma, B1 6 melanoma, and colon adenocarc inomas 26 and 38, respectively, showed inherent ly dif ferent resistance to vincrist ine (VCR) in vitro. The inherent resistance to VCR of these cell lines was related to the abil ity of the cells to accumulate VCR. Verapamil , a calcium antagonist with cor...

Abstract Peroxisomal acyl-CoA oxidase (ACOX1) deficiency is a rare autosomal recessive single enzyme characterized by hypotonia, seizures, failure to thrive, developmental delay, and neurological regression starting from approximately 3 years of age. Here, we report two siblings with ACOX1 born non-consanguineous Japanese parents. They showed mild global delay infancy began regress at 5 years 1...