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Podocytes express ADAMTS13 in normal renal cortex and in patients with thrombotic thrombocytopenic purpura

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Podocytes express ADAMTS13 in normal renal cortex and in patients with thrombotic thrombocytopenic purpura. / Manea, Minola; Kristoffersson, AnnCharlotte; Schneppenheim, Reinhard; Saleem, Moin A; Mathieson, Peter W; Mörgelin, Matthias; Björk, Peter; Holmberg, Lars; Karpman, Diana.

In: BRIT J HAEMATOL, Vol. 138, No. 5, 09.2007, p. 651-62.

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

Harvard

Manea, M, Kristoffersson, A, Schneppenheim, R, Saleem, MA, Mathieson, PW, Mörgelin, M, Björk, P, Holmberg, L & Karpman, D 2007, 'Podocytes express ADAMTS13 in normal renal cortex and in patients with thrombotic thrombocytopenic purpura', BRIT J HAEMATOL, vol. 138, no. 5, pp. 651-62. https://doi.org/10.1111/j.1365-2141.2007.06694.x

APA

Manea, M., Kristoffersson, A., Schneppenheim, R., Saleem, M. A., Mathieson, P. W., Mörgelin, M., Björk, P., Holmberg, L., & Karpman, D. (2007). Podocytes express ADAMTS13 in normal renal cortex and in patients with thrombotic thrombocytopenic purpura. BRIT J HAEMATOL, 138(5), 651-62. https://doi.org/10.1111/j.1365-2141.2007.06694.x

Vancouver

Manea M, Kristoffersson A, Schneppenheim R, Saleem MA, Mathieson PW, Mörgelin M et al. Podocytes express ADAMTS13 in normal renal cortex and in patients with thrombotic thrombocytopenic purpura. BRIT J HAEMATOL. 2007 Sep;138(5):651-62. https://doi.org/10.1111/j.1365-2141.2007.06694.x

Bibtex

@article{ecd7e0ee5d23487692a24c9b003ec1b2,
title = "Podocytes express ADAMTS13 in normal renal cortex and in patients with thrombotic thrombocytopenic purpura",
abstract = "Congenital thrombotic thrombocytopenic purpura (TTP) is associated with ADAMTS13 mutations. The major site of ADAMTS13 synthesis is the liver. Expression in other tissues, and in TTP, has not been shown. In this study, ADAMTS13 protein expression was investigated in normal kidney and in renal tissue from two TTP patients, with a compound heterozygous mutation (P353L and P457L) and a homozygous mutation (4143insA). Real-time polymerase chain reaction demonstrated ADAMTS13 mRNA in normal kidney. ADAMTS13 was detected in the glomeruli and tubuli of normal and TTP kidney using anti-ADAMTS13 antibodies. In the glomeruli, expression was localised to podocytes (as demonstrated by counterstaining with two podocyte markers) and endothelium. Similar distribution was detected in the TTP kidneys. Electron microscopy detected ADAMTS13 in podocytes, endothelium and glomerular basement membrane. Cultured human podocytes expressed ADAMTS13 mRNA and protein, and podocyte lysate exhibited von Willebrand factor-cleaving activity. Mutation expression studies of the P353L and P457L mutations showed partially impaired secretion and lower activity of the secreted mutants. Impaired secretion has previously been shown for the 4143insA mutation. Podocyte-derived ADAMTS13 may offer local protection in the high-shear microcirculation of the glomerulus. The mutations in the two TTP patients studied enabled protein expression in the podocytes but affected protease secretion.",
keywords = "ADAM Proteins, Adult, Child, Preschool, Female, Gene Expression, Glomerular Basement Membrane, Humans, Kidney Cortex, Microscopy, Electron, Mutation, Podocytes, Polymerase Chain Reaction, Purpura, Thrombotic Thrombocytopenic, RNA, Messenger",
author = "Minola Manea and AnnCharlotte Kristoffersson and Reinhard Schneppenheim and Saleem, {Moin A} and Mathieson, {Peter W} and Matthias M{\"o}rgelin and Peter Bj{\"o}rk and Lars Holmberg and Diana Karpman",
year = "2007",
month = sep,
doi = "10.1111/j.1365-2141.2007.06694.x",
language = "English",
volume = "138",
pages = "651--62",
journal = "BRIT J HAEMATOL",
issn = "0007-1048",
publisher = "Wiley-Blackwell",
number = "5",

}

RIS

TY - JOUR

T1 - Podocytes express ADAMTS13 in normal renal cortex and in patients with thrombotic thrombocytopenic purpura

AU - Manea, Minola

AU - Kristoffersson, AnnCharlotte

AU - Schneppenheim, Reinhard

AU - Saleem, Moin A

AU - Mathieson, Peter W

AU - Mörgelin, Matthias

AU - Björk, Peter

AU - Holmberg, Lars

AU - Karpman, Diana

PY - 2007/9

Y1 - 2007/9

N2 - Congenital thrombotic thrombocytopenic purpura (TTP) is associated with ADAMTS13 mutations. The major site of ADAMTS13 synthesis is the liver. Expression in other tissues, and in TTP, has not been shown. In this study, ADAMTS13 protein expression was investigated in normal kidney and in renal tissue from two TTP patients, with a compound heterozygous mutation (P353L and P457L) and a homozygous mutation (4143insA). Real-time polymerase chain reaction demonstrated ADAMTS13 mRNA in normal kidney. ADAMTS13 was detected in the glomeruli and tubuli of normal and TTP kidney using anti-ADAMTS13 antibodies. In the glomeruli, expression was localised to podocytes (as demonstrated by counterstaining with two podocyte markers) and endothelium. Similar distribution was detected in the TTP kidneys. Electron microscopy detected ADAMTS13 in podocytes, endothelium and glomerular basement membrane. Cultured human podocytes expressed ADAMTS13 mRNA and protein, and podocyte lysate exhibited von Willebrand factor-cleaving activity. Mutation expression studies of the P353L and P457L mutations showed partially impaired secretion and lower activity of the secreted mutants. Impaired secretion has previously been shown for the 4143insA mutation. Podocyte-derived ADAMTS13 may offer local protection in the high-shear microcirculation of the glomerulus. The mutations in the two TTP patients studied enabled protein expression in the podocytes but affected protease secretion.

AB - Congenital thrombotic thrombocytopenic purpura (TTP) is associated with ADAMTS13 mutations. The major site of ADAMTS13 synthesis is the liver. Expression in other tissues, and in TTP, has not been shown. In this study, ADAMTS13 protein expression was investigated in normal kidney and in renal tissue from two TTP patients, with a compound heterozygous mutation (P353L and P457L) and a homozygous mutation (4143insA). Real-time polymerase chain reaction demonstrated ADAMTS13 mRNA in normal kidney. ADAMTS13 was detected in the glomeruli and tubuli of normal and TTP kidney using anti-ADAMTS13 antibodies. In the glomeruli, expression was localised to podocytes (as demonstrated by counterstaining with two podocyte markers) and endothelium. Similar distribution was detected in the TTP kidneys. Electron microscopy detected ADAMTS13 in podocytes, endothelium and glomerular basement membrane. Cultured human podocytes expressed ADAMTS13 mRNA and protein, and podocyte lysate exhibited von Willebrand factor-cleaving activity. Mutation expression studies of the P353L and P457L mutations showed partially impaired secretion and lower activity of the secreted mutants. Impaired secretion has previously been shown for the 4143insA mutation. Podocyte-derived ADAMTS13 may offer local protection in the high-shear microcirculation of the glomerulus. The mutations in the two TTP patients studied enabled protein expression in the podocytes but affected protease secretion.

KW - ADAM Proteins

KW - Adult

KW - Child, Preschool

KW - Female

KW - Gene Expression

KW - Glomerular Basement Membrane

KW - Humans

KW - Kidney Cortex

KW - Microscopy, Electron

KW - Mutation

KW - Podocytes

KW - Polymerase Chain Reaction

KW - Purpura, Thrombotic Thrombocytopenic

KW - RNA, Messenger

U2 - 10.1111/j.1365-2141.2007.06694.x

DO - 10.1111/j.1365-2141.2007.06694.x

M3 - SCORING: Journal article

C2 - 17627784

VL - 138

SP - 651

EP - 662

JO - BRIT J HAEMATOL

JF - BRIT J HAEMATOL

SN - 0007-1048

IS - 5

ER -