Klaus‐Michael Debatin
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View article: Posttransplant B cell development and function in patients with B cell positive SCID caused by pathogenic variants in IL2RG and JAK3
Posttransplant B cell development and function in patients with B cell positive SCID caused by pathogenic variants in IL2RG and JAK3 Open
Genetic defects in IL2RG or JAK3 can cause the phenotype of severe combined immunodeficiency (SCID) with absent T- and non-functional B-lymphocytes (T-B+ SCID). B cell function and the need for immunoglobulin replacement after hematopoieti…
View article: The German Center for Child and Adolescent Health – A new structure for translational research in pediatrics shaping the health of children today and future generations
The German Center for Child and Adolescent Health – A new structure for translational research in pediatrics shaping the health of children today and future generations Open
The new German Center for Child and Adolescent Health (DZKJ) founded as part of the German Centers for Health Research provides an unprecedented and unique opportunity for internationally outstanding research that contributes to the health…
View article: Bridging the marrow: a co-culture-platform of leukemia cells and MS5-derived stromal cells or adipocytes
Bridging the marrow: a co-culture-platform of leukemia cells and MS5-derived stromal cells or adipocytes Open
In the context of acute lymphoblastic leukemia (ALL), the bone marrow microenvironment (BMM) plays a crucial role in providing pro-survival signals, as evident from the rapid spontaneous cell death observed in ex-vivo cultures of primary A…
View article: Homozygous<i>OTULIN</i>Variant Linked to OTULIN-Related Autoinflammatory Syndrome with Abscess Formation
Homozygous<i>OTULIN</i>Variant Linked to OTULIN-Related Autoinflammatory Syndrome with Abscess Formation Open
Homozygous OTU deubiquitinase with linear linkage specificity (OTULIN) variants cause OTULIN-Related Autoinflammatory Syndrome (ORAS). This disease is characterized by early-onset autoinflammation, fever, panniculitis, diarrhea, and arthri…
View article: OS07.6.A CONNEXIN-MEDIATED NETWORKS IN GLIOBLASTOMA COMMUNICATION
OS07.6.A CONNEXIN-MEDIATED NETWORKS IN GLIOBLASTOMA COMMUNICATION Open
BACKGROUND Recent data suggest that Glioblastoma (GB), a highly aggressive malignant brain tumor, mimics cellular interactions found in healthy brain structures. GB cells exhibit, among other types of interaction, extensive membrane protru…
View article: P01.07.A HOW IS A RECURRENT GLIOBLASTOMA SELECTED?
P01.07.A HOW IS A RECURRENT GLIOBLASTOMA SELECTED? Open
BACKGROUND Glioblastoma is the most common primary brain tumour in adults and one of the most lethal cancers per se. Despite aggressive therapeutic approaches, patient survival remains dismal, typically not exceeding 15 months. Standard tr…
View article: Non-Skewed X-inactivation Results in NF-κB Essential Modulator (NEMO) Δ-exon 5-autoinflammatory Syndrome (NEMO-NDAS) in a Female with Incontinentia Pigmenti
Non-Skewed X-inactivation Results in NF-κB Essential Modulator (NEMO) Δ-exon 5-autoinflammatory Syndrome (NEMO-NDAS) in a Female with Incontinentia Pigmenti Open
Purpose Genetic hypomorphic defects in X chromosomal IKBKG coding for the NF-κB essential modulator (NEMO) lead to ectodermal dysplasia and immunodeficiency in males and the skin disorder incontinentia pigmenti (IP) in females, respectivel…
View article: Venetoclax resistance in acute lymphoblastic leukemia is characterized by increased mitochondrial activity and can be overcome by co-targeting oxidative phosphorylation
Venetoclax resistance in acute lymphoblastic leukemia is characterized by increased mitochondrial activity and can be overcome by co-targeting oxidative phosphorylation Open
Deregulated apoptosis signaling is characteristic for many cancers and contributes to leukemogenesis and treatment failure in B-cell precursor acute lymphoblastic leukemia (BCP-ALL). Apoptosis is controlled by different pro- and anti-apopt…
View article: Irinotecan and temozolomide in combination with dasatinib and rapamycin versus irinotecan and temozolomide for patients with relapsed or refractory neuroblastoma (RIST-rNB-2011): a multicentre, open-label, randomised, controlled, phase 2 trial
Irinotecan and temozolomide in combination with dasatinib and rapamycin versus irinotecan and temozolomide for patients with relapsed or refractory neuroblastoma (RIST-rNB-2011): a multicentre, open-label, randomised, controlled, phase 2 trial Open
Background Neuroblastoma is the most common extracranial solid tumour in children. Relapsed or refractory neuroblastoma is associated with a poor outcome. We assessed the combination of irinotecan–temozolomide and dasatinib–rapamycin (RIST…
View article: The Alcatraz‐Strategy: a roadmap to break the connectivity barrier in malignant brain tumours
The Alcatraz‐Strategy: a roadmap to break the connectivity barrier in malignant brain tumours Open
In recent years, the discovery of functional and communicative cellular tumour networks has led to a new understanding of malignant primary brain tumours. In this review, the authors shed light on the diverse nature of cell‐to‐cell connect…
View article: The Potential Role of the Extracellular Matrix Glycoprotein Reelin in Glioblastoma Biology
The Potential Role of the Extracellular Matrix Glycoprotein Reelin in Glioblastoma Biology Open
Glioblastoma, the most common and lethal primary adult brain tumor, cannot be successfully removed surgically due to its highly invasive nature. Therapeutically, approaches must be aimed at a systemic brain disease and not merely at a tumo…
View article: Chemotherapy for a secondary malignancy nearly restores complete chimerism in an SCID-patient after HSCT
Chemotherapy for a secondary malignancy nearly restores complete chimerism in an SCID-patient after HSCT Open
For patients with inborn errors of immunity (IEI) and other inborn diseases, mixed donor chimerism is a well-accepted outcome of hematopoietic stem cell transplantation (HSCT). Cytoreductive chemotherapy for a secondary malignancy is a pot…
View article: Inhibition of Survivin Homodimerization Decreases Neuroblastoma Cell Growth
Inhibition of Survivin Homodimerization Decreases Neuroblastoma Cell Growth Open
Increased expression of BIRC5/survivin, a crucial regulator of the mitotic spindle checkpoint, is associated with poor prognosis in neuroblastoma (NB), the most common extracranial tumor of childhood. Transcriptional inhibitors of survivin…
View article: The survivin-ran inhibitor LLP-3 decreases oxidative phosphorylation, glycolysis and growth of neuroblastoma cells
The survivin-ran inhibitor LLP-3 decreases oxidative phosphorylation, glycolysis and growth of neuroblastoma cells Open
Background Neuroblastoma (NB), the most common extracranial solid malignancy in children, carries a poor prognosis in high-risk disease, thus requiring novel therapeutic approaches. Survivin is overexpressed in NB, has pro-mitotic and anti…
View article: A partial human LCK defect causes a T cell immunodeficiency with intestinal inflammation
A partial human LCK defect causes a T cell immunodeficiency with intestinal inflammation Open
Lymphocyte-specific protein tyrosine kinase (LCK) is essential for T cell antigen receptor (TCR)–mediated signal transduction. Here, we report two siblings homozygous for a novel LCK variant (c.1318C>T; P440S) characterized by T cell ly…
View article: Identification of Mir-532-5p As a Reliable miRNA Reference in Acute Lymphoblastic Leukemia
Identification of Mir-532-5p As a Reliable miRNA Reference in Acute Lymphoblastic Leukemia Open
In acute lymphoblastic leukemia (ALL) cure rates have reached >90%, however, therapy resistance and relapses occur. Hence, the major interest in leukemia research is the understanding of ALL biology and, based on that, the development o…
View article: Identification of vulnerabilities for targeting BCL-2 family members in T-Cell Acute Lymphoblastic Leukemia
Identification of vulnerabilities for targeting BCL-2 family members in T-Cell Acute Lymphoblastic Leukemia Open
T-Cell acute lymphoblastic leukemia (T-ALL) is a disease caused by the malignant transformation of T-cell lineage progenitors. With the use of intensive chemotherapies survival rates have improved, but outcomes particularly of relapsed pat…