Thrombotic Thrombocytopenic Purpura

Silverstein, Allen

doi:10.1001/archneur.1968.00470340044003

Cited by 44 publications

(2 citation statements)

References 39 publications

(1 reference statement)

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“…For instance, renal manifestations are usually more prominent in HUS than neurological ones, whereas neurological manifestations are usually more prominent in TTP than renal ones. Fever precedes TTP more commonly than it precedes HUS 75. Despite these distinctions, continued recognition of borderline or atypical cases has generated doubts about the possibility that objective criteria other than age are able to distinguish “atypical” HUS from “atypical” TTP.…”

Section: Adamts13 and Its Role In The Pathogenesis Of Thrombotic Micrmentioning

confidence: 99%

Proteolytic Processing of Von Willebrand Factor by Adamts13 and Leukocyte Proteases

Lancellotti¹,

Basso²,

Cristofaro³

2013

Mediterr J Hematol Infect Dis

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ADAMTS13 is a 190 kDa zinc protease encoded by a gene located on chromosome 9q34. This protease specifically hydrolyzes von Willebrand factor (VWF) multimers, thus causing VWF size reduction. ADAMTS13 belongs to the A Disintegrin And Metalloprotease with ThromboSpondin type 1 repeats (ADAMTS) family, involved in proteolytic processing of many matrix proteins. ADAMTS13 consists of numerous domains including a metalloprotease domain, a disintegrin domain, several thrombospondin type 1 (TSP1) repeats, a cysteine-rich domain, a spacer domain and 2 CUB (Complement c1r/c1s, sea Urchin epidermal growth factor, and Bone morphogenetic protein) domains. ADAMTS13 cleaves a single peptide bond (Tyr1605-Met1606) in the central A2 domain of the VWF molecule. This proteolytic cleavage is essential to reduce the size of ultra-large VWF polymers, which, when exposed to high shear stress in the microcirculation, are prone to form with platelets clumps, which cause severe syndromes called thrombotic microangiopathies (TMAs). In this review, we a) discuss the current knowledge of structure-function aspects of ADAMTS13 and its involvement in the pathogenesis of TMAs, b) address the recent findings concerning proteolytic processing of VWF multimers by different proteases, such as the leukocyte-derived serine and metallo-proteases and c) indicate the direction of future investigations.

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Section: Adamts13 and Its Role In The Pathogenesis Of Thrombotic Micrmentioning

confidence: 99%

Proteolytic Processing of Von Willebrand Factor by Adamts13 and Leukocyte Proteases

Lancellotti¹,

Basso²,

Cristofaro³

2013

Mediterr J Hematol Infect Dis

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“…Abnormalities were present in 35 of 38 (92%) at the time of admission and appeared later in the remainder of patients. The neurologic manifestations of TTP were well summarized by Silverstein in 1966 [7]. He surveyed over 300 case reports, selecting 168 for neurologic review.…”

Section: Symptoms Signs and Presenting Statusmentioning

confidence: 99%