• Publications
  • Influence
Sequencing in an Assembly Line with Blocking to Minimize Cycle Time
We consider an assembly line with m stations in series having finite capacity buffers. Blocking occurs when buffers are full. There are M different types of products to be assembled, each with itsExpand
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Biotinidase deficiency: the enzymatic defect in late-onset multiple carboxylase deficiency.
Late-onset multiple carboxylase deficiency is characterized clinically by skin rash, alopecia, seizures and ataxia and occasionally by candidiasis and developmental delay. Biochemically, theseExpand
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Biotinidase deficiency: “if you have to have an inherited metabolic disease, this is the one to have”
  • B. Wolf
  • Medicine
  • Genetics in Medicine
  • 5 January 2012
Biotinidase recycles the vitamin biotin. Biotinidase deficiency is an autosomal recessively inherited neurocutaneous disorder. The symptoms of the disorder can be successfully treated or prevented byExpand
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Clinical issues and frequent questions about biotinidase deficiency.
  • B. Wolf
  • Medicine
  • Molecular genetics and metabolism
  • 1 May 2010
Biotinidase deficiency is a biotin-responsive, inherited neurocutaneous disorder. The disorder is readily treatable and is screened for in the newborn period. Over the years since the discovery ofExpand
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Biotinylation of histones by human serum biotinidase: assessment of biotinyl-transferase activity in sera from normal individuals and children with biotinidase deficiency.
Serum biotinidase has biotinyl-transferase activity in addition to biocytin hydrolase activity. A sensitive assay for biotinyl-transferase activity was developed based on the transfer of biotin fromExpand
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Biotinidase Deficiency a
There are two major types of multiple carboxylasedeficiency (MCD), the neonatal form in which the usual presenting symptoms are vomiting, lethargy and hypotonia, and the late-onset or juvenile formExpand
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  • Open Access
Phenotypic variation in biotinidase deficiency.
Biotinidase deficiency is the usual biochemical defect in biotin-responsive late-onset multiple carboxylase deficiency. We reviewed the clinical features of six patients with the enzyme deficiencyExpand
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The neurology of biotinidase deficiency.
  • B. Wolf
  • Biology, Medicine
  • Molecular genetics and metabolism
  • 1 September 2011
Biotinidase deficiency is an autosomal recessively inherited metabolic disorder in which the enzyme, biotinidase, is defective and the vitamin, biotin, is not recycled. Individuals with biotinidaseExpand
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Disorders of Biotin Metabolism
There are several inherited disorders that affect biotin metabolism; these are holocarboxylase synthetase (HCS) deficiency, biotinidase deficiency, and biotin-responsive basal ganglia disease. EachExpand
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Clinical heterogeneity in 80 home-reared children with cri du chat syndrome.
A population of 80 home-reared children with cri du chat syndrome was investigated to document the clinical heterogeneity of the syndrome and to analyze the factors influencing the severity of theExpand
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