In Manitoba, Canada, the overall incidence of Severe Combined Immunodeficiency (SCID) is three-fold higher than the national average, with SCID overrepresented in two population groups: Mennonites and First Nations of Northern Cree ancestries.
O. Jilkina +5 more
doaj +1 more source
Recent Developments in NSG and NRG Humanized Mouse Models for Their Use in Viral and Immune Research
Humanized mouse models have been widely used in virology, immunology, and oncology in the last decade. With advances in the generation of knockout mouse strains, it is now possible to generate animals in which human immune cells or human tissue can be ...
Maksym Kitsera +2 more
doaj +1 more source
Resveratrol given intraperitoneally does not inhibit the growth of high-risk t(4;11) acute lymphoblastic leukemia cells in a NOD/SCID mouse model. [PDF]
The efficacy of resveratrol as a preventive agent against the growth of t(4;11) acute lymphoblastic leukemia (ALL) was evaluated in NOD.CB17-Prkdcscid/J mice engrafted with the human t(4;11) ALL SEM cell line.
Ducore, Jonathan M +5 more
core +1 more source
Effective therapeutic targeting of CTNNB1‐mutant hepatoblastoma with WNTinib
WNTinib, a Wnt/CTNNB1 inhibitor, was tested in hepatoblastoma (HB) experimental models. It delayed tumor growth and improved survival in CTNNB1‐mutant in vivo models. In organoids, WNTinib outperformed cisplatin and showed enhanced efficacy in combination therapy, supporting its potential as a targeted treatment for CTNNB1‐mutated HB.
Ugne Balaseviciute +17 more
wiley +1 more source
The C Terminus of Ku80 activates the DNA-dependent protein kinase catalytic subunit [PDF]
Ku is a heterodimeric protein with double-stranded DNA end-binding activity that operates in the process of nonhomologous end joining. Ku is thought to target the DNA-dependent protein kinase (DNA-PK) complex to the DNA and, when DNA bound, can interact ...
Jeggo, P A +4 more
core +2 more sources
A nonsense variant of reactivation gene-1 leads to Omenn syndrome in a Pakistani family
OBJECTIVE: To conduct clinical and genetic analysis in a patient with severe combined immunodeficiency disease (SCID). METHODS: A 3.5-year-old female patient with chronic diarrhea, fever, and failure to thrive was examined at the Pakistan Institute of ...
Zara Khalid +5 more
doaj +1 more source
Introducing Newborn Screening for Severe Combined Immunodeficiency—The New Zealand Experience
Screening for severe combined immunodeficiency (SCID) was added to the New Zealand national newborn screening programme in December 2017. Documentation pertaining to the application to add SCID to the panel and screening results over the first three ...
Natasha Heather +5 more
doaj +1 more source
Pharmacologic ascorbate (vitamin C) increases ROS, disrupts cellular metabolism, and induces DNA damage in CRPC cells. These effects sensitize tumors to PARP inhibition, producing synergistic growth suppression with olaparib in vitro and significantly delayed tumor progression in vivo. Pyruvate rescue confirms ROS‐dependent activity.
Nicolas Gordon +13 more
wiley +1 more source
Impaired bone marrow homing of cytokine-activated CD34+ cells in the NOD/SCID model [PDF]
The reduced engraftment potential of hematopoietic stem/progenitor cells (HSPCs) after exposure to cytokines may be related to the impaired homing ability of actively cycling cells. We tested this hypothesis by quantifying the short-term horning of human
Ahmed, F +8 more
core
Establishing Newborn Screening for SCID in the USA: Experience in California
Newborn screening for severe combined immunodeficiency (SCID) has developed from the realization that infants affected with SCID require prompt diagnosis and treatment to avoid fatal infectious complications.
Jennifer M. Puck, Andrew R. Gennery
doaj +1 more source

