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Increased expression of lysosomal acid phosphatase in CLN3-defective cells and mouse brain tissue.

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Increased expression of lysosomal acid phosphatase in CLN3-defective cells and mouse brain tissue. / Pohl, Sandra; Mitchison, Hannah M; Kohlschütter, Alfried; van Diggelen, Otto; Braulke, Thomas; Storch, Stephan.

In: J NEUROCHEM, Vol. 103, No. 6, 6, 2007, p. 2177-2188.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Pohl, S, Mitchison, HM, Kohlschütter, A, van Diggelen, O, Braulke, T & Storch, S 2007, 'Increased expression of lysosomal acid phosphatase in CLN3-defective cells and mouse brain tissue.', J NEUROCHEM, vol. 103, no. 6, 6, pp. 2177-2188. <http://www.ncbi.nlm.nih.gov/pubmed/17868323?dopt=Citation>

APA

Pohl, S., Mitchison, H. M., Kohlschütter, A., van Diggelen, O., Braulke, T., & Storch, S. (2007). Increased expression of lysosomal acid phosphatase in CLN3-defective cells and mouse brain tissue. J NEUROCHEM, 103(6), 2177-2188. [6]. http://www.ncbi.nlm.nih.gov/pubmed/17868323?dopt=Citation

Vancouver

Pohl S, Mitchison HM, Kohlschütter A, van Diggelen O, Braulke T, Storch S. Increased expression of lysosomal acid phosphatase in CLN3-defective cells and mouse brain tissue. J NEUROCHEM. 2007;103(6):2177-2188. 6.

Bibtex

@article{149688424f514ae69c3d22700e52aab6,
title = "Increased expression of lysosomal acid phosphatase in CLN3-defective cells and mouse brain tissue.",
abstract = "Juvenile neuronal ceroid lipofuscinosis (Batten disease) is a neurodegenerative disorder caused by defective function of the lysosomal membrane glycoprotein CLN3. The activity of the lysosomal acid phosphatase (LAP/ACP2) was found to be significantly increased in the cerebellum and brain stem of Cln3-targeted mice during the early stages of postnatal life. Histochemical localization studies revealed an increased LAP/ACP2 staining intensity in neurons of the cerebral cortex of 48-week-old Cln3-targeted mice as compared with controls. Additionally, the expression of another lysosomal membrane protein LAMP-2 was increased in all brain areas. Knockdown of CLN3 expression in HeLa cells by RNA interference also resulted in increased LAP/ACP2 and LAMP-2 expression. Finally in fibroblasts of two juvenile neuronal ceroid lipofuscinosis patients elevated levels of LAP/ACP2 were found. Both activation of gene transcription and increased protein half-life appear to contribute to increased LAP/ACP2 protein expression in CLN3-deficient cells. The data suggest that lysosomal dysfunction and accumulation of storage material require increased biogenesis of LAP/ACP2 and LAMP-2 positive membranes which makes LAP/ACP2 suitable as biomarker of Batten disease.",
author = "Sandra Pohl and Mitchison, {Hannah M} and Alfried Kohlsch{\"u}tter and {van Diggelen}, Otto and Thomas Braulke and Stephan Storch",
year = "2007",
language = "Deutsch",
volume = "103",
pages = "2177--2188",
journal = "J NEUROCHEM",
issn = "0022-3042",
publisher = "Wiley-Blackwell",
number = "6",

}

RIS

TY - JOUR

T1 - Increased expression of lysosomal acid phosphatase in CLN3-defective cells and mouse brain tissue.

AU - Pohl, Sandra

AU - Mitchison, Hannah M

AU - Kohlschütter, Alfried

AU - van Diggelen, Otto

AU - Braulke, Thomas

AU - Storch, Stephan

PY - 2007

Y1 - 2007

N2 - Juvenile neuronal ceroid lipofuscinosis (Batten disease) is a neurodegenerative disorder caused by defective function of the lysosomal membrane glycoprotein CLN3. The activity of the lysosomal acid phosphatase (LAP/ACP2) was found to be significantly increased in the cerebellum and brain stem of Cln3-targeted mice during the early stages of postnatal life. Histochemical localization studies revealed an increased LAP/ACP2 staining intensity in neurons of the cerebral cortex of 48-week-old Cln3-targeted mice as compared with controls. Additionally, the expression of another lysosomal membrane protein LAMP-2 was increased in all brain areas. Knockdown of CLN3 expression in HeLa cells by RNA interference also resulted in increased LAP/ACP2 and LAMP-2 expression. Finally in fibroblasts of two juvenile neuronal ceroid lipofuscinosis patients elevated levels of LAP/ACP2 were found. Both activation of gene transcription and increased protein half-life appear to contribute to increased LAP/ACP2 protein expression in CLN3-deficient cells. The data suggest that lysosomal dysfunction and accumulation of storage material require increased biogenesis of LAP/ACP2 and LAMP-2 positive membranes which makes LAP/ACP2 suitable as biomarker of Batten disease.

AB - Juvenile neuronal ceroid lipofuscinosis (Batten disease) is a neurodegenerative disorder caused by defective function of the lysosomal membrane glycoprotein CLN3. The activity of the lysosomal acid phosphatase (LAP/ACP2) was found to be significantly increased in the cerebellum and brain stem of Cln3-targeted mice during the early stages of postnatal life. Histochemical localization studies revealed an increased LAP/ACP2 staining intensity in neurons of the cerebral cortex of 48-week-old Cln3-targeted mice as compared with controls. Additionally, the expression of another lysosomal membrane protein LAMP-2 was increased in all brain areas. Knockdown of CLN3 expression in HeLa cells by RNA interference also resulted in increased LAP/ACP2 and LAMP-2 expression. Finally in fibroblasts of two juvenile neuronal ceroid lipofuscinosis patients elevated levels of LAP/ACP2 were found. Both activation of gene transcription and increased protein half-life appear to contribute to increased LAP/ACP2 protein expression in CLN3-deficient cells. The data suggest that lysosomal dysfunction and accumulation of storage material require increased biogenesis of LAP/ACP2 and LAMP-2 positive membranes which makes LAP/ACP2 suitable as biomarker of Batten disease.

M3 - SCORING: Zeitschriftenaufsatz

VL - 103

SP - 2177

EP - 2188

JO - J NEUROCHEM

JF - J NEUROCHEM

SN - 0022-3042

IS - 6

M1 - 6

ER -