BAG3 antibody
- Known as:
- BAG3 (anti-)
- Catalog number:
- orb101835
- Product Quantity:
- EUR
- Category:
- -
- Supplier:
- Biorbyt biorb
- Gene target:
- BAG3 antibody
Related genes to: BAG3 antibody
- Gene:
- BAG3 NIH gene
- Name:
- BCL2 associated athanogene 3
- Previous symbol:
- -
- Synonyms:
- -
- Chromosome:
- 10q26.11
- Locus Type:
- gene with protein product
- Date approved:
- 1999-04-23
- Date modifiied:
- 2019-04-23
Related products to: BAG3 antibody
Related articles to: BAG3 antibody
- Heat shock protein family B member 8 (HSPB8) is a chaperone involved in the chaperone-assisted selective autophagy (CASA) complex. HSPB8 in conjunction with cochaperone BAG3, promotes autophagy-mediated removal of misfolded proteins associated with various neurodegenerative diseases. Mutations in , previously associated with Charcot Marie Tooth disease type 2L, have recently been linked to an autosomal dominant rimmed vacuolar myopathy (MFM13), and is considered a multisystem proteinopathy. Patients have distal and proximal limb girdle myopathy with muscle biopsy showing fatty replacement, endomysial fibrosis, and rimmed vacuoles leading to muscle atrophy and early demise. We have demonstrated reduced expression of HSPB8, altered autophagy and TDP-43 accumulation in patient fibroblasts. Using CRISPR technology, we generated a knock-in 8 mouse model of the c.515dupC hot spot frameshift variant to study disease pathology. Overexpressed murine frameshift mutant (c.515dupC, fs) displays insolubility and aggregation propensity in Murine Neuroblastoma X Spinal Cord 34 (NSC-34) cells. Mutant mice developed late-onset muscle weakness beginning at 15 months. Muscle biochemical analyses revealed reduced HSPB8 levels, increased TDP-43, and altered autophagy markers, partially recapitulating the human phenotype. Fiber type analysis, neuromuscular junction integrity, and motor neurons show mild myopathy without neurodegeneration. Given the lack of available treatments, we evaluated trehalose, a natural disaccharide that induces HSPB8 and enhances autophagy. Administration of 2% trehalose in drinking water improves motor performance, restores HSPB8 expression, and ameliorates autophagic and TDP-43 pathology in mutant mice. These findings support the value of our preclinical models for translational studies, and autophagy enhancement as a potential therapeutic strategy for HSPB8-related myopathy. - Source: PubMed
Publication date: 2026/08/09
Shmara AlyaaWeiss LanGromova AnastasiaTedesco BarbaraPal PallabiKostalnick GenieBoock VictoriaBassett ElizabethParera SebastianCheng ChengTa LacLee JonathanPanchagatti ArjunMohanty EshaneeVu JillianLa Spada Albert RPoletti AngeloKimonis Virginia - Ménière disease (MD) is a chronic inner ear disorder characterized by recurrent vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural fullness. Diagnosis is clinical, and no validated peripheral biomarkers are available. Although endolymphatic hydrops is a hallmark, its poor correlation with symptom severity suggests broader systemic mechanisms. - Source: PubMed
Publication date: 2026/06/22
Chiarella GiuseppeCuda GiovanniParrotta Elvira IScalise StefaniaCarvelli SimonaScarpa AlfonsoScaramuzzino LuanaCovello RaffaeleMartines FrancescoNeri GiampieroFetoni Anna RViola Pasquale - Dilated cardiomyopathy (DCM) is a leading cause of heart failure with notable sex differences in susceptibility and progression. Although sarcomere mutations such as cardiac actin ACTC1 p.T126I contribute to familial DCM, the in vivo effects and sex-specific consequences remain unclear. We generated a zebrafish model carrying the orthologous Acta1b p.T126I mutation and conducted longitudinal, sex-stratified analyses of cardiac function, morphology and gene expression. Mutants showed variable onset of cardiac dysfunction, with progressive DCM, pericardial effusion, ventricular dilation, and reduced survival in adults. Female mutants exhibited earlier and sustained diastolic dysfunction, greater cardiac remodeling and significantly lower survival compared to males, revealing pronounced sexual dimorphism. Molecular profiling at a pre-symptomatic stage identified upregulation of nppb, downregulation of hypertrophic transcription factors (gata4, mef2ca), and sex-specific alterations in calcium handling genes (serca2, pln1, slc8a1a) and proteostasis regulators (hsf1, bag3). Older stages demonstrated a variable shift of individuals' gene expression to cardiac remodeling and decompensation. These findings demonstrate that the Acta1b p.T126I mutation drives progressive, sex-specific DCM in zebrafish, highlighting biological sex as a critical modifier of sarcomeric cardiomyopathy progression and targeted therapy development. - Source: PubMed
Publication date: 2026/08/07
Prill KendalDawson John F - - Source: PubMed
Publication date: 2026/08/04
Manzo PaolaCammarota Anna LisaBasile AnnaDel Papa NicolettaIervolino MatteoUnfer VittorioTurco Maria CaterinaDe Marco Margot - Resistance exercise (RE) improves strength and muscle mass, with multiple benefits for human health. However, intense RE also induces acute myofibrillar damage. The molecular mechanisms that preserve, mark, degrade, and restore damaged proteins to keep skeletal muscle working under RE are incompletely understood. Based on repeated sampling of human skeletal muscle, we show here that acute, repeated and interrupted RE induce dynamic changes of the protein landscape associated with the sarcomeric cytoskeleton. These changes correlate with changes in phosphorylation indicative of adaptation and deadaptation signaling footprints. Regulation mainly affects the protein network linked to the muscle maintenance protein BAG3, which includes mechanosensory proteins, small heat shock proteins, and a lipid droplet associated protein. All network components exhibit altered phosphorylation and increased cytoskeletal association after damaging RE. Moreover, network components cooperate to recognize strained skeletal muscle structures and mediate their degradation through chaperone-assisted selective autophagy (CASA). Our study thus identifies key regulators of skeletal muscle homeostasis in humans. - Source: PubMed
Publication date: 2026/07/28
Kuppusamy MaithreyanJacko DanielGupta YaminiSieger SandroSchaaf KirillMatijass MartinBersiner KätheZacher JonasBonini SaraCosenza-Contreras Miguelvan der Ven PeterBloch WilhelmFürst Dieter OWinter DominicHöhfeld JörgHuesgen Pitter FGehlert Sebastian