PMID- 15869605
OWN - NLM
STAT- MEDLINE
DCOM- 20050819
LR  - 20230829
IS  - 1538-7933 (Print)
IS  - 1538-7836 (Linking)
VI  - 3
IP  - 5
DP  - 2005 May
TI  - Cloning, expression and functional characterization of the full-length murine 
      ADAMTS13.
PG  - 1064-73
AB  - Functional deficiency or absence of the human von Willebrand factor 
      (VWF)-cleaving protease (VWF-cp), recently termed ADAMTS13, has been shown to 
      cause acquired and congenital thrombotic thrombocytopenic purpura (TTP), 
      respectively. As a first step towards developing a small animal model of TTP, we 
      have cloned the complete (non-truncated) murine Adamts13 gene from BALB/c mice 
      liver poly A+ mRNA. Murine ADAMTS13 is a 1426-amino-acid protein with a high 
      homology and similar structural organization to the human ortholog. Transient 
      expression of the murine Adamts13 cDNA in HEK 293 cells yielded a protein with a 
      molecular weight of approximately 180 kDa which degraded recombinant murine VWF 
      (rVWF) in a dose-dependent manner. The cleavage products of murine rVWF had the 
      expected size of 140 and 170 kDa. Murine ADAMTS13 was inhibited by EDTA and the 
      plasma from a TTP patient.
FAU - Bruno, K
AU  - Bruno K
AD  - Baxter BioScience, Vienna, Austria.
FAU - Volkel, D
AU  - Volkel D
FAU - Plaimauer, B
AU  - Plaimauer B
FAU - Antoine, G
AU  - Antoine G
FAU - Pable, S
AU  - Pable S
FAU - Motto, D G
AU  - Motto DG
FAU - Lemmerhirt, H L
AU  - Lemmerhirt HL
FAU - Dorner, F
AU  - Dorner F
FAU - Zimmermann, K
AU  - Zimmermann K
FAU - Scheiflinger, F
AU  - Scheiflinger F
LA  - eng
PT  - Journal Article
PL  - England
TA  - J Thromb Haemost
JT  - Journal of thrombosis and haemostasis : JTH
JID - 101170508
RN  - 0 (Amino Acids)
RN  - 0 (DNA Primers)
RN  - 0 (DNA, Complementary)
RN  - 0 (RNA, Messenger)
RN  - 0 (Recombinant Proteins)
RN  - 0 (von Willebrand Factor)
RN  - 24937-83-5 (Poly A)
RN  - 63231-63-0 (RNA)
RN  - 9G34HU7RV0 (Edetic Acid)
RN  - EC 3.4.24.- (ADAM Proteins)
RN  - EC 3.4.24.- (ADAMTS13 protein, mouse)
RN  - EC 3.4.24.- (Metalloendopeptidases)
RN  - EC 3.4.24.87 (ADAMTS13 Protein)
RN  - EC 3.4.24.87 (ADAMTS13 protein, human)
SB  - IM
MH  - ADAM Proteins
MH  - ADAMTS13 Protein
MH  - Amino Acid Sequence
MH  - Amino Acids/chemistry
MH  - Animals
MH  - Blotting, Northern
MH  - Blotting, Western
MH  - Cell Line
MH  - Cloning, Molecular
MH  - DNA Primers/chemistry
MH  - DNA, Complementary/metabolism
MH  - Databases, Genetic
MH  - Dose-Response Relationship, Drug
MH  - Edetic Acid/pharmacology
MH  - Humans
MH  - Metalloendopeptidases/chemistry/*genetics/*metabolism
MH  - Mice
MH  - Mice, Inbred BALB C
MH  - Models, Genetic
MH  - Molecular Sequence Data
MH  - Phylogeny
MH  - Poly A/metabolism
MH  - Polymerase Chain Reaction
MH  - Protein Binding
MH  - Protein Structure, Tertiary
MH  - Purpura, Thrombotic Thrombocytopenic/blood
MH  - RNA/metabolism
MH  - RNA, Messenger/metabolism
MH  - Recombinant Proteins/chemistry
MH  - Sequence Homology, Amino Acid
MH  - Tissue Distribution
MH  - Transfection
MH  - von Willebrand Factor/metabolism
EDAT- 2005/05/05 09:00
MHDA- 2005/08/20 09:00
CRDT- 2005/05/05 09:00
PHST- 2005/05/05 09:00 [pubmed]
PHST- 2005/08/20 09:00 [medline]
PHST- 2005/05/05 09:00 [entrez]
AID - S1538-7836(22)15965-9 [pii]
AID - 10.1111/j.1538-7836.2005.01246.x [doi]
PST - ppublish
SO  - J Thromb Haemost. 2005 May;3(5):1064-73. doi: 10.1111/j.1538-7836.2005.01246.x.