A sensitive radioassay for biotinidase activity: deficient activity in tissues of serum biotinidase-deficient individuals

Barry Wolf; Julie Secor McVoy

doi:10.1016/0009-8981(83)90286-3

A sensitive radioassay for biotinidase activity: deficient activity in tissues of serum biotinidase-deficient individuals

Barry Wolf^*, Julie Secor McVoy

^*Corresponding author for this work

Pediatrics

Research output: Contribution to journal › Article › peer-review

37 Scopus citations

Abstract

A new, sensitive radioassay for the determination of biotinidase activity was developed which measures the release of [¹⁴C-carboxylj-p-aminobenzoate fromN-biotinyl-[¹⁴C-carboxyl]-p-aminobenzoate. Biotinidase activity in serum from normal individuals is comparable to that determined by the colorimetric assay, but the radioassay is approximately 100 times more sensitive. Biotinidase deficiency was confirmed in the serum of patients who were previously shown to have reduced activities by the colorimetric assay. Although biotinidase activity was not detectable in extracts of normal peripheral blood leukocytes and fibroblasts using the colorimetric assay, activities could be measured by the radioassay. Using this method we demonstrated deficient biotinidase activity in extracts of leukocytes and fibroblasts from affected patients.

Original language	English (US)
Pages (from-to)	275-281
Number of pages	7
Journal	Clinica Chimica Acta
Volume	135
Issue number	3
DOIs	https://doi.org/10.1016/0009-8981(83)90286-3
State	Published - Dec 30 1983

Funding

The authors thank Drs. R.J. Allen, S.I. Goodman, C.L. Kien and M. Batshaw for sending us fibroblasts and blood from patients, Sharon Suchy for her helpful discussions and suggestions, and Terry Mayo for her excellent secretarial assistance. This work was supported by grants from the National Institutes of Health (AM 25675) and from the National Foundation-March of Dimes (6-342). B.W. is the recipient of an NIH Research Career Development Award (AM 00677). This is paper No. 207 from the Department of Human Genetics of the Medical College of Virginia.

ASJC Scopus subject areas

Biochemistry
Clinical Biochemistry
Biochemistry, medical

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title = "A sensitive radioassay for biotinidase activity: deficient activity in tissues of serum biotinidase-deficient individuals",

abstract = "A new, sensitive radioassay for the determination of biotinidase activity was developed which measures the release of [14C-carboxylj-p-aminobenzoate fromN-biotinyl-[14C-carboxyl]-p-aminobenzoate. Biotinidase activity in serum from normal individuals is comparable to that determined by the colorimetric assay, but the radioassay is approximately 100 times more sensitive. Biotinidase deficiency was confirmed in the serum of patients who were previously shown to have reduced activities by the colorimetric assay. Although biotinidase activity was not detectable in extracts of normal peripheral blood leukocytes and fibroblasts using the colorimetric assay, activities could be measured by the radioassay. Using this method we demonstrated deficient biotinidase activity in extracts of leukocytes and fibroblasts from affected patients.",

author = "Barry Wolf and McVoy, {Julie Secor}",

note = "Funding Information: The authors thank Drs. R.J. Allen, S.I. Goodman, C.L. Kien and M. Batshaw for sending us fibroblasts and blood from patients, Sharon Suchy for her helpful discussions and suggestions, and Terry Mayo for her excellent secretarial assistance. This work was supported by grants from the National Institutes of Health (AM 25675) and from the National Foundation-March of Dimes (6-342). B.W. is the recipient of an NIH Research Career Development Award (AM 00677). This is paper No. 207 from the Department of Human Genetics of the Medical College of Virginia.",

year = "1983",

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doi = "10.1016/0009-8981(83)90286-3",

language = "English (US)",

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T1 - A sensitive radioassay for biotinidase activity

T2 - deficient activity in tissues of serum biotinidase-deficient individuals

AU - Wolf, Barry

AU - McVoy, Julie Secor

N1 - Funding Information: The authors thank Drs. R.J. Allen, S.I. Goodman, C.L. Kien and M. Batshaw for sending us fibroblasts and blood from patients, Sharon Suchy for her helpful discussions and suggestions, and Terry Mayo for her excellent secretarial assistance. This work was supported by grants from the National Institutes of Health (AM 25675) and from the National Foundation-March of Dimes (6-342). B.W. is the recipient of an NIH Research Career Development Award (AM 00677). This is paper No. 207 from the Department of Human Genetics of the Medical College of Virginia.

PY - 1983/12/30

Y1 - 1983/12/30

N2 - A new, sensitive radioassay for the determination of biotinidase activity was developed which measures the release of [14C-carboxylj-p-aminobenzoate fromN-biotinyl-[14C-carboxyl]-p-aminobenzoate. Biotinidase activity in serum from normal individuals is comparable to that determined by the colorimetric assay, but the radioassay is approximately 100 times more sensitive. Biotinidase deficiency was confirmed in the serum of patients who were previously shown to have reduced activities by the colorimetric assay. Although biotinidase activity was not detectable in extracts of normal peripheral blood leukocytes and fibroblasts using the colorimetric assay, activities could be measured by the radioassay. Using this method we demonstrated deficient biotinidase activity in extracts of leukocytes and fibroblasts from affected patients.

AB - A new, sensitive radioassay for the determination of biotinidase activity was developed which measures the release of [14C-carboxylj-p-aminobenzoate fromN-biotinyl-[14C-carboxyl]-p-aminobenzoate. Biotinidase activity in serum from normal individuals is comparable to that determined by the colorimetric assay, but the radioassay is approximately 100 times more sensitive. Biotinidase deficiency was confirmed in the serum of patients who were previously shown to have reduced activities by the colorimetric assay. Although biotinidase activity was not detectable in extracts of normal peripheral blood leukocytes and fibroblasts using the colorimetric assay, activities could be measured by the radioassay. Using this method we demonstrated deficient biotinidase activity in extracts of leukocytes and fibroblasts from affected patients.

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A sensitive radioassay for biotinidase activity: deficient activity in tissues of serum biotinidase-deficient individuals

Abstract

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