We report biochemical, morphological and neuroradiological findings in a 40-year-old woman affected with type I sialidosis. The clinical symptoms, consisting of a cerebellar syndrome, were first noted at the age of 17 years. The macular cherry-red spot was first observed after 23 years of disease. A CT scan performed at 21 years of age showed enlargement of the fourth ventricle. Nuclear magneti...

We describe an approach to model biochemical reaction networks at the level of promotion-inhibition circuitry through a class of stochastic spin models that depart from the usual chemical kinetics setup and includes spatial and temporal density fluctuations in a most natural way. A particular but otherwise generally applicable choice for the microscopic transition rates of the models also makes...

The sporulation-specific enterotoxin of Clostridium perfringens type A, which is the toxin active in human food poisoning, has been purified from extracts of sporulating cells. Highly purified enterotoxin was obtained by treatment of crude cell extract with ribonuclease for 30 min, followed by sequential chromatography on Sephadex G-100, Cellex T cellulose, and hydroxylapatite. Recovery was 65 ...

BACKGROUND Renal amyloidosis results from protein misfolding and leads to progressive renal insufficiency. Few data are available concerning the relevance of the histomorphological patterns and the dynamics of the disease process. METHODS Cases of renal amyloidosis in native kidney biopsies (n = 203) were retrospectively evaluated for the pattern of amyloid distribution, the extent of glomeru...

mycoplasma ovipneumoniae is an important respiratory pathogen of sheep across the world. in the present study nasal swab samples (n=778) were processed and 51isolates of m. ovipneumoniae were identified through biochemical tests and pcr. difference in the isolation rate among districts (n=12), age groups (n=3), breads (n=6) was assessed. similarly the isolate rate among healthy (n=513) and resp...

Diabetes mellitus is considered to be one of the most common chronic diseases worldwide, and recognized as one of the leading causes of morbidity and mortality (American Diabetes Association, 2010). It has been reported that the prevalence of diabetes mellitus will increase from 6% to over 10% in the next decade (Rosen et al., 2001). According to the World Health Organization in 2000, a total o...

We previously reported the isolation and functional characterization of seven adenovirus type 5 (Ad5) DNA-binding protein (DBP) point mutants (Quinn, C. O., and Kitchingman, G. R. (1986) J. Virol. 60, 653-661). Six of the seven mutants were defective in their ability to help adeno-associated virus replicate its DNA. To determine the level at which the mutations affect this function of the DBP, ...

We analyzed tissue and cells from a stationary and a rapidly growing hyperplastic callus from a patient with osteogenesis imperfecta (OI) type IV and compared the results with those of compact bone and skin fibroblasts of an age-matched control. Collagen and protein contents per cell were low in the callus tissues and collagen I and III were overmodified as evidenced by an elevated level of hyd...

Biochemical reaction systems may be viewed as discrete event processes characterized by a number of states and state transitions. These systems may be modeled as state transition systems with transitions representing individual reaction events. Since they often involve a large number of interactions, it can be difficult to construct such a model for a system, and since the resulting state-level...

Cytochromes c are metalloproteins that function in electron transfer reactions and contain a heme moiety covalently attached via thioether linkages between the co-factor and a CXXCH motif in the protein. Covalent attachment of the heme group occurs on the positive side of all energy-transducing membranes (bacterial periplasm, mitochondrial intermembrane space and thylakoid lumen) and requires m...