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Rare and hereditary disorders
Batten disease
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Publications and studies (974)
- Laboratory or animal researchiNo research in humans yet. Promising in a test tube or in mice unfortunately means nothing for patients. Defective synaptic transmission causes disease signs in a mouse model of juvenile neuronal ceroid lipofuscinosis. (2017/11/14) ♡
- Role of the Lysosomal Membrane Protein, CLN3, in the Regulation of Cathepsin D Activity. (2017/11/01) ♡
- Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Manganese in manganism, Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis, and Batten disease: A narrative review. (2017/11/01) ♡
- Commentary or editorialiAn expert's opinion or commentary, not new research. Manganese, manganism and other neurodegenerative diseases: Is it a cause of concern? (2017/11/01) ♡
- Glial cells are functionally impaired in juvenile neuronal ceroid lipofuscinosis and detrimental to neurons. (2017/10/17) ♡
- Complete Genome Sequence of Peste des Petits Ruminants Virus from Georgia, 2016. (2017/10/12) ♡
- Proteomic Analysis of Brain and Cerebrospinal Fluid from the Three Major Forms of Neuronal Ceroid Lipofuscinosis Reveals Potential Biomarkers. (2017/10/06) ♡
- Development of a Novel Reverse Transcription Loop-Mediated Isothermal Amplification Assay for the Rapid Detection of African Horse Sickness Virus. (2017/10/01) ♡
- Laboratory or animal researchiNo research in humans yet. Promising in a test tube or in mice unfortunately means nothing for patients. Conditional loss of progranulin in neurons is not sufficient to cause neuronal ceroid lipofuscinosis-like neuropathology in mice. (2017/10/01) ♡
- Proteomic mapping of differentially vulnerable pre-synaptic populations identifies regulators of neuronal stability in vivo. (2017/09/29) ♡
- Lipidomic and Transcriptomic Basis of Lysosomal Dysfunction in Progranulin Deficiency. (2017/09/12) ♡
- Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Linking mitochondrial dysfunction to neurodegeneration in lysosomal storage diseases. (2017/09/01) ♡
- Laboratory or animal researchiNo research in humans yet. Promising in a test tube or in mice unfortunately means nothing for patients. Loss of Cln5 causes altered neurogenesis in a mouse model of a childhood neurodegenerative disorder. (2017/09/01) ♡
- Pharmacological Effects on Ceroid Lipofuscin and Neuronal Structure in Cln3 (∆ex7/8) Mouse Brain Cultures. (2017/09/01) ♡
- Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Exacerbating and reversing lysosomal storage diseases: from yeast to humans. (2017/08/25) ♡
- Fingolimod and Teriflunomide Attenuate Neurodegeneration in Mouse Models of Neuronal Ceroid Lipofuscinosis. (2017/08/02) ♡
- Commentary or editorialiAn expert's opinion or commentary, not new research. Progress toward Fulfilling the Potential of Immunomodulation in Childhood Neurodegeneration? (2017/08/02) ♡
- Efficacy of Eplerenone in the Management of Mineralocorticoid Excess in Men With Metastatic Castration-resistant Prostate Cancer Treated With Abiraterone Without Prednisone. (2017/08/01) ♡
- CLN5 is cleaved by members of the SPP/SPPL family to produce a mature soluble protein. (2017/08/01) ♡
- Clinical trialiResearch in patients, without randomization between groups. Useful, but less certain than a randomized trial. Phenotype and natural history of variant late infantile ceroid-lipofuscinosis 5. (2017/08/01) ♡
- Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. Muscle ceroid lipofuscin-like deposits in a patient with corticobasal syndrome due to a progranulin mutation. (2017/08/01) ♡
- Commentary or editorialiAn expert's opinion or commentary, not new research. The value of a comprehensive natural history in late infantile CLN5 disease. (2017/08/01) ♡
- Photosensitivity is an early marker of neuronal ceroid lipofuscinosis type 2 disease. (2017/08/01) ♡
- Primary fibroblasts from CSPα mutation carriers recapitulate hallmarks of the adult onset neuronal ceroid lipofuscinosis. (2017/07/24) ♡
- Thermal Stability as a Determinant of AAV Serotype Identity. (2017/07/24) ♡
- Synergistic effects of treating the spinal cord and brain in CLN1 disease. (2017/07/18) ♡
- A Basic ApoE-Based Peptide Mediator to Deliver Proteins across the Blood-Brain Barrier: Long-Term Efficacy, Toxicity, and Mechanism. (2017/07/05) ♡
- Aberrant adhesion impacts early development in a Dictyostelium model for juvenile neuronal ceroid lipofuscinosis. (2017/07/04) ♡
- in vivo localization of the neuronal ceroid lipofuscinosis proteins, CLN3 and CLN7, at endogenous expression levels. (2017/07/01) ♡
- Loss of Cln3 impacts protein secretion in the social amoeba Dictyostelium. (2017/07/01) ♡
- Laboratory or animal researchiNo research in humans yet. Promising in a test tube or in mice unfortunately means nothing for patients. Gene Therapy of Adult Neuronal Ceroid Lipofuscinoses with CRISPR/Cas9 in Zebrafish. (2017/07/01) ♡
- Purkinje Cells Are More Vulnerable to the Specific Depletion of Cathepsin D Than to That of Atg7. (2017/07/01) ♡
- Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Cerliponase Alfa: First Global Approval. (2017/07/01) ♡
- Retinal function in patients with the neuronal ceroid lipofuscinosis phenotype. (2017/07/01) ♡
- Calcineurin/NFAT Signaling in Activated Astrocytes Drives Network Hyperexcitability in Aβ-Bearing Mice. (2017/06/21) ♡
- Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Safety and potential efficacy of gemfibrozil as a supportive treatment for children with late infantile neuronal ceroid lipofuscinosis and other lipid storage disorders. (2017/06/17) ♡
- Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. NCLs and ER: A stressful relationship. (2017/06/01) ♡
- Multiplex Tandem Mass Spectrometry Enzymatic Activity Assay for Newborn Screening of the Mucopolysaccharidoses and Type 2 Neuronal Ceroid Lipofuscinosis. (2017/06/01) ♡
- Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. Long-term follow-up of two siblings with adult-onset neuronal ceroid lipofuscinosis, Kufs type A. (2017/06/01) ♡
- Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Induced pluripotent stem cell models of lysosomal storage disorders. (2017/06/01) ♡
- Impaired prosaposin lysosomal trafficking in frontotemporal lobar degeneration due to progranulin mutations. (2017/05/25) ♡
- Laboratory or animal researchiNo research in humans yet. Promising in a test tube or in mice unfortunately means nothing for patients. Retinal Degeneration In A Mouse Model Of CLN5 Disease Is Associated With Compromised Autophagy. (2017/05/09) ♡
- Laboratory or animal researchiNo research in humans yet. Promising in a test tube or in mice unfortunately means nothing for patients. A tailored mouse model of CLN2 disease: A nonsense mutant for testing personalized therapies. (2017/05/02) ♡
- Ocular morphology and function in juvenile neuronal ceroid lipofuscinosis (CLN3) in the first decade of life. (2017/05/01) ♡
- Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Inherited diseases caused by mutations in cathepsin protease genes. (2017/05/01) ♡
- Laboratory or animal researchiNo research in humans yet. Promising in a test tube or in mice unfortunately means nothing for patients. Gemfibrozil, food and drug administration-approved lipid-lowering drug, increases longevity in mouse model of late infantile neuronal ceroid lipofuscinosis. (2017/05/01) ♡
- Proteomics insights into infantile neuronal ceroid lipofuscinosis (CLN1) point to the involvement of cilia pathology in the disease. (2017/05/01) ♡
- Induced Pluripotent Stem Cells Derived from a CLN5 Patient Manifest Phenotypic Characteristics of Neuronal Ceroid Lipofuscinoses. (2017/05/01) ♡
- Individuals with progranulin haploinsufficiency exhibit features of neuronal ceroid lipofuscinosis. (2017/04/12) ♡
- Laboratory or animal researchiNo research in humans yet. Promising in a test tube or in mice unfortunately means nothing for patients. Age-dependent alterations in neuronal activity in the hippocampus and visual cortex in a mouse model of Juvenile Neuronal Ceroid Lipofuscinosis (CLN3). (2017/04/01) ♡
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