By the same authors

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A Specific Activity-Based Probe to Monitor Family GH59 Galactosylceramidase, the Enzyme Deficient in Krabbe Disease

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A Specific Activity-Based Probe to Monitor Family GH59 Galactosylceramidase, the Enzyme Deficient in Krabbe Disease. / Marques, André R. A. ; Willems, Lianne I.; Herrera Moro, Daniela; Florea, Bogdan I.; Scheij, Saskia; Ottenhoff, Roelof; van Roomen, Cindy P.A.A.; Verhoek, Marri; Nelson, Jessica K.; Kallemeijn, Wouter W.; Biela-Banas, Anna; Martin, Olivier R.; Cachón‐González , M. Begoña; Kim, Nee Na; Cox, Timothy M.; Boot, Rolf G.; Overkleeft, Herman S.; Aerts, Johannes M F G.

In: Chembiochem, Vol. 18, No. 4, 31.01.2017, p. 402-412.

Research output: Contribution to journalArticlepeer-review

Harvard

Marques, ARA, Willems, LI, Herrera Moro, D, Florea, BI, Scheij, S, Ottenhoff, R, van Roomen, CPAA, Verhoek, M, Nelson, JK, Kallemeijn, WW, Biela-Banas, A, Martin, OR, Cachón‐González , MB, Kim, NN, Cox, TM, Boot, RG, Overkleeft, HS & Aerts, JMFG 2017, 'A Specific Activity-Based Probe to Monitor Family GH59 Galactosylceramidase, the Enzyme Deficient in Krabbe Disease', Chembiochem, vol. 18, no. 4, pp. 402-412. https://doi.org/10.1002/cbic.201600561

APA

Marques, A. R. A., Willems, L. I., Herrera Moro, D., Florea, B. I., Scheij, S., Ottenhoff, R., van Roomen, C. P. A. A., Verhoek, M., Nelson, J. K., Kallemeijn, W. W., Biela-Banas, A., Martin, O. R., Cachón‐González , M. B., Kim, N. N., Cox, T. M., Boot, R. G., Overkleeft, H. S., & Aerts, J. M. F. G. (2017). A Specific Activity-Based Probe to Monitor Family GH59 Galactosylceramidase, the Enzyme Deficient in Krabbe Disease. Chembiochem, 18(4), 402-412. https://doi.org/10.1002/cbic.201600561

Vancouver

Marques ARA, Willems LI, Herrera Moro D, Florea BI, Scheij S, Ottenhoff R et al. A Specific Activity-Based Probe to Monitor Family GH59 Galactosylceramidase, the Enzyme Deficient in Krabbe Disease. Chembiochem. 2017 Jan 31;18(4):402-412. https://doi.org/10.1002/cbic.201600561

Author

Marques, André R. A. ; Willems, Lianne I. ; Herrera Moro, Daniela ; Florea, Bogdan I. ; Scheij, Saskia ; Ottenhoff, Roelof ; van Roomen, Cindy P.A.A. ; Verhoek, Marri ; Nelson, Jessica K. ; Kallemeijn, Wouter W. ; Biela-Banas, Anna ; Martin, Olivier R. ; Cachón‐González , M. Begoña ; Kim, Nee Na ; Cox, Timothy M. ; Boot, Rolf G. ; Overkleeft, Herman S. ; Aerts, Johannes M F G. / A Specific Activity-Based Probe to Monitor Family GH59 Galactosylceramidase, the Enzyme Deficient in Krabbe Disease. In: Chembiochem. 2017 ; Vol. 18, No. 4. pp. 402-412.

Bibtex - Download

@article{f786c0a0e5084890b003ab284391a49b,
title = "A Specific Activity-Based Probe to Monitor Family GH59 Galactosylceramidase, the Enzyme Deficient in Krabbe Disease",
abstract = "Galactosylceramidase (GALC) is the lysosomal β-galactosidase responsible for the hydrolysis of galactosylceramide. Inherited deficiency in GALC causes Krabbe disease, a devastating neurological disorder characterized by accumulation of galactosylceramide and its deacylated counterpart, the toxic sphingoid base galactosylsphingosine (psychosine). We report the design and application of a fluorescently tagged activity-based probe (ABP) for the sensitive and specific labeling of active GALC molecules from various species. The probe consists of a β-galactopyranose-configured cyclophellitol-epoxide core, conferring specificity for GALC, equipped with a BODIPY fluorophore at C6 that allows visualization of active enzyme in cells and tissues. Detection of residual GALC in patient fibroblasts holds great promise for laboratory diagnosis of Krabbe disease. We further describe a procedure for in situ imaging of active GALC in murine brain by intra-cerebroventricular infusion of the ABP. In conclusion, this GALC-specific ABP should find broad applications in diagnosis, drug development, and evaluation of therapy for Krabbe disease.",
keywords = "beta-galactosidase, fluorescent probes, galactosylceramidase, hydrolase, Krabbe disease",
author = "Marques, {Andr{\'e} R. A.} and Willems, {Lianne I.} and {Herrera Moro}, Daniela and Florea, {Bogdan I.} and Saskia Scheij and Roelof Ottenhoff and {van Roomen}, {Cindy P.A.A.} and Marri Verhoek and Nelson, {Jessica K.} and Kallemeijn, {Wouter W.} and Anna Biela-Banas and Martin, {Olivier R.} and Cach{\'o}n‐Gonz{\'a}lez, {M. Bego{\~n}a} and Kim, {Nee Na} and Cox, {Timothy M.} and Boot, {Rolf G.} and Overkleeft, {Herman S.} and Aerts, {Johannes M F G}",
note = "This is an author-produced version of the published paper. Uploaded in accordance with the publisher{\textquoteright}s self-archiving policy. Further copying may not be permitted; contact the publisher for details",
year = "2017",
month = jan,
day = "31",
doi = "10.1002/cbic.201600561",
language = "English",
volume = "18",
pages = "402--412",
journal = "Chembiochem",
issn = "1439-4227",
publisher = "Wiley-VCH Verlag",
number = "4",

}

RIS (suitable for import to EndNote) - Download

TY - JOUR

T1 - A Specific Activity-Based Probe to Monitor Family GH59 Galactosylceramidase, the Enzyme Deficient in Krabbe Disease

AU - Marques, André R. A.

AU - Willems, Lianne I.

AU - Herrera Moro, Daniela

AU - Florea, Bogdan I.

AU - Scheij, Saskia

AU - Ottenhoff, Roelof

AU - van Roomen, Cindy P.A.A.

AU - Verhoek, Marri

AU - Nelson, Jessica K.

AU - Kallemeijn, Wouter W.

AU - Biela-Banas, Anna

AU - Martin, Olivier R.

AU - Cachón‐González , M. Begoña

AU - Kim, Nee Na

AU - Cox, Timothy M.

AU - Boot, Rolf G.

AU - Overkleeft, Herman S.

AU - Aerts, Johannes M F G

N1 - This is an author-produced version of the published paper. Uploaded in accordance with the publisher’s self-archiving policy. Further copying may not be permitted; contact the publisher for details

PY - 2017/1/31

Y1 - 2017/1/31

N2 - Galactosylceramidase (GALC) is the lysosomal β-galactosidase responsible for the hydrolysis of galactosylceramide. Inherited deficiency in GALC causes Krabbe disease, a devastating neurological disorder characterized by accumulation of galactosylceramide and its deacylated counterpart, the toxic sphingoid base galactosylsphingosine (psychosine). We report the design and application of a fluorescently tagged activity-based probe (ABP) for the sensitive and specific labeling of active GALC molecules from various species. The probe consists of a β-galactopyranose-configured cyclophellitol-epoxide core, conferring specificity for GALC, equipped with a BODIPY fluorophore at C6 that allows visualization of active enzyme in cells and tissues. Detection of residual GALC in patient fibroblasts holds great promise for laboratory diagnosis of Krabbe disease. We further describe a procedure for in situ imaging of active GALC in murine brain by intra-cerebroventricular infusion of the ABP. In conclusion, this GALC-specific ABP should find broad applications in diagnosis, drug development, and evaluation of therapy for Krabbe disease.

AB - Galactosylceramidase (GALC) is the lysosomal β-galactosidase responsible for the hydrolysis of galactosylceramide. Inherited deficiency in GALC causes Krabbe disease, a devastating neurological disorder characterized by accumulation of galactosylceramide and its deacylated counterpart, the toxic sphingoid base galactosylsphingosine (psychosine). We report the design and application of a fluorescently tagged activity-based probe (ABP) for the sensitive and specific labeling of active GALC molecules from various species. The probe consists of a β-galactopyranose-configured cyclophellitol-epoxide core, conferring specificity for GALC, equipped with a BODIPY fluorophore at C6 that allows visualization of active enzyme in cells and tissues. Detection of residual GALC in patient fibroblasts holds great promise for laboratory diagnosis of Krabbe disease. We further describe a procedure for in situ imaging of active GALC in murine brain by intra-cerebroventricular infusion of the ABP. In conclusion, this GALC-specific ABP should find broad applications in diagnosis, drug development, and evaluation of therapy for Krabbe disease.

KW - beta-galactosidase

KW - fluorescent probes

KW - galactosylceramidase

KW - hydrolase

KW - Krabbe disease

UR - http://www.scopus.com/inward/record.url?scp=85011041183&partnerID=8YFLogxK

U2 - 10.1002/cbic.201600561

DO - 10.1002/cbic.201600561

M3 - Article

C2 - 28000364

AN - SCOPUS:85011041183

VL - 18

SP - 402

EP - 412

JO - Chembiochem

JF - Chembiochem

SN - 1439-4227

IS - 4

ER -