ADAMTS13
- Known as:
- ADAMTS13
- Catalog number:
- 001165A
- Product Quantity:
- 250ul
- Category:
- -
- Supplier:
- ABM
- Gene target:
- ADAMTS13
Related genes to: ADAMTS13
- Gene:
- ADAMTS13 NIH gene
- Name:
- ADAM metallopeptidase with thrombospondin type 1 motif 13
- Previous symbol:
- C9orf8
- Synonyms:
- VWFCP, TTP, vWF-CP, FLJ42993, MGC118899, MGC118900, DKFZp434C2322
- Chromosome:
- 9q34.2
- Locus Type:
- gene with protein product
- Date approved:
- 1999-08-23
- Date modifiied:
- 2019-04-23
Related products to: ADAMTS13
Related articles to: ADAMTS13
- Thrombotic microangiopathy (TMA) with associated nephrotic syndrome is a unique and rare condition that is infrequently described in pediatrics. - Source: PubMed
Publication date: 2026/04/28
Fisher DorHaskin OrlyLandau DanielBorovitz YaelRinat ChoniAlfandary Hadas - Thrombocytopenic purpura (TTP) is a hematologic emergency that may occur with PD-L1 immunotherapy. New or worsening anemia and thrombocytopenia in patients started on PD-L1 inhibitors should raise suspicion for TTP and prompt workup for thrombotic microangiopathy. - Source: PubMed
Publication date: 2026/03/12
Ke JasonChong Albert CHsu RobertMartynova AnastasiaWald-Dickler NoahD'Souza AnishkaPiatek CarolineIn Gino K - Deficiency of ADAMTS13 caused by autoantibodies leads to the thrombotic microangiopathy immune thrombotic thrombocytopenic purpura (iTTP). Corticosteroids have long been utilized in the management of patients with iTTP, both in the acute setting and continued after clinical remission has been achieved, usually tapered over a period of weeks. However, with recent advances in the field, the landscape of steroid utilization is changing, particularly since the mid-2010s with the advent of caplacizumab and anti-cluster of differentiation 20 (CD20) directed therapy in iTTP. Though steroids are generally well tolerated, many patients are at risk of adverse events related to corticosteroid use, such as patients with diabetes mellitus, hypertension, and cardiovascular disease. There are no uniform guidelines regarding optimal steroid formulations, dosages, or duration of treatment. Modern treatment modalities target more specific aspects of the pathophysiology of iTTP, leading to speculation that steroid-free regimens may become the new standard of care. Despite this, because of the favorable adverse event profile of steroids and the modest benefit they provide, steroid use is still widespread for patients with iTTP. As such, there is a need for further studies regarding corticosteroid dosing; duration of treatment with corticosteroids; adverse events associated specifically with the use of corticosteroids; and whether, how, or in what context corticosteroids can safely be omitted or replaced with another treatment modality in the care of patients with iTTP. In this Mini-Review, we will explore the historical reasoning and data supporting the use of corticosteroids in iTTP and the shifting role of steroids in the modern era. - Source: PubMed
Publication date: 2025/12/18
Halkidis Konstantine - Von Willebrand factor (VWF) and ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, 13) are linked to dementia risk, and limited evidence suggests ()-ε4 alters VWF release. This study assessed whether baseline VWF and ADAMTS13 levels predict neurodegeneration and cognitive decline and evaluated effect modification by carriership. - Source: PubMed
Publication date: 2026/04/20
Adegboye Hailey ASun YunyiZhang PanpanLiu DandanKhan Omair ATanriverdi KahramanRisitano AntoninaLibby JuliaPatterson Khiry LArul Albert BOliver Nekesa CWhitaker Marsalas DJanve Vaibhav ADumitrescu Logan CPechman Kimberly RShashikumar NiranjanaBolton Corey JDavis L TaylorLandman Bennett AFreedman Jane ERobinson Renã A SHohman Timothy JJefferson Angela L - Thrombotic microangiopathy (TMA) is a rare but life-threatening complication in patients with cancer and can be difficult to distinguish from other treatment-related toxicities. We report the case of an eight-year-old girl with metastatic Wilms' tumor and a solitary kidney after left nephrectomy who developed biopsy-confirmed TMA following intensive multimodal therapy. She received multiple lines of chemotherapy, including actinomycin D, vincristine, cyclophosphamide, doxorubicin, and carboplatin, as well as radiotherapy to the bilateral lungs, nephrectomy bed, and whole abdomen. Several months into treatment, she developed progressive renal insufficiency with rising serum creatinine, microscopic hematuria, proteinuria, and subsequent extra-renal manifestations, including heart failure and new-onset seizures. Laboratory evaluation showed acute kidney injury, and renal biopsy demonstrated focal fibrin thrombi with mesangiolysis consistent with acute TMA, alongside mild acute tubular injury and non-specific immunofluorescence findings. In the absence of access to ADAMTS13 and complement genetic testing, chemotherapy-induced TMA, most likely related to cumulative exposure to carboplatin or doxorubicin in the context of prior radiotherapy, was considered the leading diagnosis. Withdrawal of chemotherapy was followed by partial renal recovery. This case highlights the need for a high index of suspicion for drug-induced TMA in pediatric oncology patients who develop new renal dysfunction and neurological or cardiac symptoms during or after exposure to multiple potentially endothelial-toxic agents. It also highlights the central role of renal biopsy in diagnosis and the importance of early recognition and treatment modification to preserve renal function, particularly in children with limited nephron reserve. - Source: PubMed
Publication date: 2026/03/11
Jamil MaryamKhan Bilal Shahzad AzamRashid AqeelaShaheen Najma