Glomerular Complement Factor H-Related Protein 5 (FHR5) Is Highly Prevalent in C3 Glomerulopathy and Associated With Renal Impairment.

Introduction: Therapeutic agents that target complement are increasingly available for glomerular diseases. However, the mechanisms linking glomerular complement deposition with inflammation and damage are incompletely understood. Complement factor H-related protein 5 (FHR5) interacts with complement C3 and is considered to promote activation.

SU-E-T-479: Skin Dose from Flattening Filter Free Beams: A Monte Carlo Investigation.

Purpose: Flattening filter free (FFF) beams in radiotherapy have advantages such as shorter treatment delivery time and lower out-of-field dose compared with conventional flattened beams. This study investigates in detail the skin dose induced by FFF beams from a TrueBeam accelerator (Varian Medical Systems, Palo Alto, CA) using Monte Carlo method.

Methods: Phase space files generated using real geometry of a TrueBeam accelerator above the jaws, were used as the input radiation source files in beam simulation for various field sizes using BEAMnrc.

Characterisation of the FAM69 family of cysteine-rich endoplasmic reticulum proteins.

The FAM69 family of cysteine-rich type II transmembrane proteins comprises three members in all vertebrates except fish, and orthologues with a conserved structure are present throughout metazoa. All three murine FAM69 proteins (FAM69A, FAM69B, FAM69C) localise to the endoplasmic reticulum (ER) in cultured cells, probably via N-terminal di-arginine motifs. Mammalian FAM69A is ubiquitously expressed, FAM69B is strongly expressed in the brain and in peripheral endothelial cells, and FAM69C in the brain and eye.

SIRT2 inhibition achieves neuroprotection by decreasing sterol biosynthesis.

Huntington's disease (HD), an incurable neurodegenerative disorder, has a complex pathogenesis including protein aggregation and the dysregulation of neuronal transcription and metabolism. Here, we demonstrate that inhibition of sirtuin 2 (SIRT2) achieves neuroprotection in cellular and invertebrate models of HD. Genetic or pharmacologic inhibition of SIRT2 in a striatal neuron model of HD resulted in gene expression changes including significant down-regulation of RNAs responsible for sterol biosynthesis.

Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease.

Huntingtin proteolysis has been implicated in the molecular pathogenesis of Huntington disease (HD). Despite an intense effort, the identity of the pathogenic smallest N-terminal fragment has not been determined. Using a panel of anti-huntingtin antibodies, we employed an unbiased approach to generate proteolytic cleavage maps of mutant and wild-type huntingtin in the HdhQ150 knock-in mouse model of HD.