Prodynorphin and Proenkephalin in Cerebrospinal Fluid of Sporadic Creutzfeldt-Jakob Disease

Article Clinical Neurology

Cerebrospinal Fluid Levels of Prodynorphin-Derived Peptides Are Decreased in Huntington's Disease

M. H. D. Rami Al Shweiki et al.

Summary: PDYN-derived peptides were found to be substantially decreased in the CSF of HD patients, while NfL levels were elevated, indicating that PDYN-derived peptides may be a more specific biomarker for HD.

MOVEMENT DISORDERS (2021)

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Article Clinical Neurology

Proenkephalin Decreases in Cerebrospinal Fluid with Symptom Progression of Huntington's Disease

Valter Niemela et al.

Summary: Identifying molecular changes in Huntington's disease is crucial for therapy development. Mass-spectrometry analysis revealed differences in protein expression between HD patients and controls, with PENK levels potentially serving as a marker for the state of medium spiny neurons in HD.

MOVEMENT DISORDERS (2021)

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Review Clinical Neurology

Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease

Peter Hermann et al.

Summary: Sporadic Creutzfeldt-Jakob disease is a fatal neurodegenerative disease caused by misfolded prion proteins. Its diagnosis relies on a combination of neuropsychiatric symptoms, CSF markers, and imaging. The development of assays like RT-QuIC has improved diagnostic accuracy.

LANCET NEUROLOGY (2021)

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Review Neurosciences

Cerebrospinal Fluid and Blood Neurofilament Light Chain Protein in Prion Disease and Other Rapidly Progressive Dementias: Current State of the Art

Samir Abu-Rumeileh et al.

Summary: Rapidly progressive dementia refers to conditions causing rapid neurological deterioration associated with cognitive decline, such as Creutzfeldt-Jakob disease. The neurofilament light chain protein could serve as a biofluid marker in differential diagnosis and prognostic assessment in RPDs.

FRONTIERS IN NEUROSCIENCE (2021)

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Review Biochemistry & Molecular Biology

Endogenous Opioid Peptides and Alternatively Spliced Mu Opioid Receptor Seven Transmembrane Carboxyl-Terminal Variants

Anna Abrimian et al.

Summary: Endogenous opioid peptides, including enkephalins, dynorphins, and beta-endorphin, act through MOR, DOR, and KOR receptors, playing important roles in analgesia and various biological processes. The OPRM1 gene undergoes extensive pre-mRNA splicing, generating multiple splice variants, with the 7TM C-terminal variants playing a key role in the pharmacological functions of endogenous opioid peptides.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2021)

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Article Clinical Neurology

Phenotypic diversity of genetic Creutzfeldt-Jakob disease: a histo-molecular-based classification

Simone Baiardi et al.

Summary: The current classification of sporadic Creutzfeldt-Jakob disease (sCJD) includes six major clinicopathological subtypes based on the physicochemical properties of the protease-resistant core of the pathologic prion protein (PrPSc), defining two major PrPSc types (1 and 2), and the codon 129 genotype on the prion protein gene (PRNP). The research analyzed 208 individuals affected by genetic CJD (gCJD), identifying six major groups based on the combination of PrPSc type and codon 129 genotype on PRNP mutated allele. The phenotypic variability of gCJD is mostly consistent with that previously found in sCJD.

ACTA NEUROPATHOLOGICA (2021)

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Article Clinical Neurology

Diagnostic and prognostic performance of CSF α-synuclein in prion disease in the context of rapidly progressive dementia

Andrea Mastrangelo et al.

Summary: In patients with prion disease, CSF alpha-synuclein levels were significantly higher compared to other subtypes, but had lower diagnostic value than CSF total tau or 14-3-3. Additionally, CSF alpha-synuclein was significantly associated with survival in both the prion cohort and the most common clinicopathological subtypes.

ALZHEIMER'S & DEMENTIA: DIAGNOSIS, ASSESSMENT & DISEASE MONITORING (2021)

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Article Biochemistry & Molecular Biology