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ambroxol (ambroxol, Diffucaps)

✓ Approved

Adare Pharma Solutions · 小分子 · 小分子

什么是 ambroxol?

ambroxol 是一种小分子,由Adare Pharma Solutions研发。该药已获批,用于治疗相关适应症,给药途径:Oral (PO)。

药物档案

商品名ambroxol, Diffucaps
公司Adare Pharma Solutions
药物类别小分子
给药途径Oral (PO)
状态Approved

治疗适应症

ambroxol 针对 1 个适应症,涉及 1 个治疗领域。

治疗领域疾病/病症分期
Respiratory, thoracic and mediastinal disordersBronchitis chronic✓ Approved

相关研究文献

PubMedMetabolic brain disease2026-09-09

Therapeutics candidates and repurposing strategies to target parkinson's disease pathology: current evidence and future directions.

Dhanush Yarava Y, Nizamuddin N D ND, Neelima Satrasala S, Kalpana S S et al.

Parkinson's disease (PD) is the second most prevalent neurodegenerative disorder worldwide, affecting over 8.5 million individuals globally. Its pathophysiology is multifactorial, encompassing progressive loss of dopaminergic neurons in the substantia nigra, accumulation of misfolded alpha-synuclein, mitochondrial dysfunction, oxidative stress, neuroinflammation, and impaired proteostasis. Despite decades of research, current treatments remain predominantly symptomatic, with levodopa and dopamine replacement therapies failing to halt neurodegeneration. Given the high cost and low success rate of de novo drug development, both repurposed approved drugs and mechanistically targeted investigational therapeutics have emerged as promising approaches as a strategically rational alternative that leverages established safety profiles, known pharmacokinetics, and abbreviated regulatory pathways. This review aimed to comprehensively examine the current evidence for repurposed drugs, clinically investigated therapeutic candidates, and repositioning strategies targeting the principal pathological hallmarks of Parkinson's disease: (1) alpha-synuclein aggregation and propagation, (2) oxidative stress, (3) neuroinflammation, (4) mitochondrial dysfunction, (5) lysosomal and proteasomal dysfunction, and (6) dopaminergic neurodegeneration. The review further sought to identify recurring translational challenges, propose mechanistic frameworks for rational combination therapy, and outline future directions for trial design and biomarker integration. A narrative review of published preclinical studies, clinical trials, and recent literature was conducted, focusing on repurposed compounds with mechanistic plausibility and evidence of CNS penetration relevant to PD pathology. Evidence from Phase 1-3 clinical trials and post-mortem neuropathological analyses was synthesised across each pathological hallmark. Compounds were evaluated for mechanistic specificity, pharmacokinetic suitability, and clinical translation status. Across six pathological hallmarks, repurposed agents showed disease-relevant activity, with variable clinical translation. Ambroxol, a GCase pharmacological chaperone, achieved ~ 30-34% brain penetration, increased cerebrospinal fluid alpha-synuclein in a Phase 2 trial (n = 17) regardless of GBA mutation status, and is now in Phase 3 (ASPro-PD, n = 330). N-acetylcysteine restored depleted brain glutathione, scavenged reactive oxygen species, preserved VMAT2 and tyrosine hydroxylase expression, and improved motor outcomes, with positive dopamine transporter imaging in clinical studies. Doxycycline, a BBB-penetrant antibiotic, suppressed microglial MMP-3 and MMP-9 and downregulated TNF-α, IL-1β, iNOS, and COX-2 in preclinical PD models. Metformin and its mitochondria-targeted analog Mito-Met activated AMPK signalling and reversed mitochondrial dysfunction in PD models; however, Mito-Q10 failed in a one-year clinical trial, highlighting that ~ 70% of dopaminergic neurons are already lost at symptom onset, making intervention timing a decisive variable. For proteostatic dysfunction, the USP14 inhibitor IU1 enhanced both proteasomal and autophagic flux, though neurotoxicity above 200 µM limits its therapeutic window; BIIB122, a selective LRRK2 inhibitor, achieved CNS target engagement in Phase 1/2 trials, but its Phase 3 LIGHTHOUSE study was terminated, with Phase 2b LUMA ongoing. In dopaminergic neurodegeneration, GLP-1 receptor agonists produced divergent outcomes: Exenatide-PD3 (n = 231) showed no benefit over placebo, whereas LixiPark demonstrated reduced motor progression in early-stage PD, revealing that mechanistic equivalence does not guarantee clinical equivalence across heterogeneous patient populations. Drug repurposing represents a viable and strategically advantageous approach to targeting PD pathophysiology. Repurposed compounds, including ambroxol, N-acetylcysteine, doxycycline, metformin, and GLP-1 receptor agonists, demonstrate engagement with disease-relevant mechanisms and, in several cases, preliminary evidence of clinical activity. However, a recurring translational challenge is the timing of therapeutic intervention: with approximately 70% of dopaminergic neurons already lost at symptom onset, even mechanistically sound agents may fail if administered too late. Divergent GLP-1 trial outcomes underscore that mechanistic equivalence does not guarantee clinical equivalence, and that disease-stage heterogeneity is a critical confounder. Future priorities must include: (i) precision stratification by genetic profile (e.g., GBA and LRRK2 variants); (ii) combination therapies targeting multiple interconnected hallmarks simultaneously; (iii) development of validated fluid and neuroimaging biomarkers for early-stage patient selection; and (iv) adaptive trial designs that accommodate heterogeneity across the PD population. Integration of computational tools network pharmacology, machine learning, and systems biology with biomarker-defined clinical enrichment will be essential for accelerating repurposed candidates toward meaningful disease modification.

PMID 42714656
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PubMedNeuromolecular medicine2026-09-01

Rebalancing α-Synuclein Clearance: Novel Therapeutic Frontiers in Parkinson's Disease.

Jabeen Maryam M, Hameed Huma H, Ahmad Syed Muhammad SM, Mushtaq Tehreem T et al.

Parkinson's disease (PD), the second most common neurodegenerative condition, develops because of abnormal protein misfolding and aggregation of α-synuclein with its subsequent intercellular spread. Such pathological changes lead to disruption of neuronal homeostasis and contribute to neuronal degeneration. During normal conditions, α-synuclein clearance is controlled by different types of lysosomal degradation, namely, macro autophagy, chaperone-mediated autophagy (CMA), micro autophagy, and the ubiquitin-proteasome system. Malfunction of these systems results in increased α-synuclein secretion due to exosome-dependent, direct, and damage-induced mechanisms, which, in turn, promotes enhanced intercellular propagation, inflammation, mitochondrial dysfunction, blood-brain barrier leakage, and neuronal cell death. Although several approaches targeting α-synuclein clearance have shown biological activity in preclinical or early clinical studies, consistent disease-modifying efficacy has not yet been established, owing to challenges including target specificity, blood brain barrier penetration, biological heterogeneity, and the limited sensitivity of clinical endpoints. Recent research indicates that successful treatment is more related to restoring the balance of these two processes than to manipulating one of them.In this review, it is proposed that a systems-level approach can be taken where PD is understood as a disease characterized by the imbalance in proteostasis. Potential treatment modalities include small molecules targeting lysosome function (ambroxol, rapamycin, TFEB activators), CMA enhancers, gene therapy, and antibodies against extracellular α-synuclein. Furthermore, new modalities like molecular glue degraders, allostery-based stabilization of α-synuclein tetramers, engineered decoy particles, and bispecific antibodies represent some other possible routes towards multimodal disease modification.

PMID 42675202
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PubMedMolecular biology reports2026-08-21

Glucocerebrosidase dysfunction in GBA1 carriers: insights from blood and macrophage analyses.

Nikolaev Mikhail M, Kopytova Alena A, Izyumchenko Artem A, Senkevich Konstantin K et al.

Mutations in the GBA1 gene, which encodes the lysosomal enzyme glucocerebrosidase (GCase), are the most common genetic factor associated with Parkinson's disease (PD). These mutations are classified as "severe" or "mild" based on the residual GCase activity. This study aimed to compare the biochemical characteristics of peripheral blood and macrophages derived from peripheral blood mononuclear cells (PBMC-derived macrophages) from PD patients with GBA1 mutations (GBA1-PD) and healthy GBA1 mutation carriers (GBA1-carriers). 31 GBA1-PD patients, 24 GBA1-carriers and 247 controls were enrolled. We assessed GCase activity, levels of the lysosphingolipid hexosylsphingosine (HexSph) and the proteins GCase, alpha-synuclein, and cathepsin D as well as GCase translocation to lysosomes in PBMC-derived macrophages. Biochemical analysis of PBMC-derived macrophages revealed similar impairments in GCase activity, lysosomal translocation, elevated HexSph and alpha-synuclein, and decreased cathepsin D in both GBA1-PD and GBA1-carriers compared to controls. However, when the mutations were divided according to their severity, almost all differences were only observed in the carriers of "severe" mutations. The ratio of GCase activity to HexSph level in blood significantly differed between GBA1-PD and GBA1-carriers. Additionally, we retrospectively analyzed data on GCase activity and HexSph level in blood of GBA1-PD patient during the presymptomatic period prior motor symptom onset, following oral ambroxol treatment (1200 mg per day). Biochemical alterations in GCase function are linked to GBA1 mutations independent of PD status, with "severe" mutations notably impacting lysosomal function and multiple cellular processes. We propose the GCase activity to HexSph ratio as a novel biomarker for disease progression and monitoring therapeutic response.

PMID 42627573
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PubMedFrontiers in pediatrics2026-08-18

Analysis of the interventional effects of ambroxol hydrochloride and ontelukast sodium combined with azithromycin on immune function and C-reactive protein serum expression in children with severe Mycoplasma pneumoniae pneumonia.

Liu Xueru X, Chen Xiang X, Ye Shan S

The aim of the study was to investigate the clinical effects of ambroxol hydrochloride and montelukast sodium combined with azithromycin on immune function, inflammatory cytokines, and pulmonary function in children with severe Mycoplasma pneumoniae pneumonia (SMPP). A retrospective cohort study was conducted on 103 children with SMPP treated at Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science & Technology, from February 2023 to February 2025. On the basis of different treatment regimens recorded in the electronic medical record system, the children were divided into a control group (n = 50, montelukast sodium combined with azithromycin) and an observation group (n = 53, ambroxol hydrochloride added to the control regimen). To address the selection bias inherent in the retrospective design, a propensity score analysis based on inverse probability of treatment weighting (IPTW) was performed; a logistic regression model incorporating age, sex, disease duration, only-child status, paternal and maternal education levels, and place of residence as covariates was used to derive the propensity scores, and stabilized weights were applied so that all 103 patients contributed to the weighted analysis without exclusion. Clinical data were collected and compared, including baseline characteristics, symptoms and signs (time for cough to subside, time for body temperature to normalize, time for sputum to disappear, and time for rales to disappear), immune function (CD3+, CD4+, CD8+, CD4+/CD8+), inflammatory cytokines [tumor necrosis factor-alpha (TNF-α), interferon-gamma (IFN-γ), interleukin-2 (IL-2), interleukin-4 (IL-4), and C-reactive protein (CRP)], pulmonary function [forced expiratory volume in the first second (FEV1), forced vital capacity (FVC), and maximal mid-expiratory flow (MMEF)], clinical efficacy, and adverse reactions. The total clinical efficacy rate was pre-specified as the primary outcome, and all remaining endpoints were treated as secondary or exploratory; a Bonferroni-corrected significance threshold of α = 0.003 was applied across the 16 secondary continuous outcomes. Effect sizes (Cohen's d) and 95% confidence intervals (CIs) were computed for all between-group comparisons. After treatment, the observation group showed significantly greater improvement in symptoms and signs, immune function, inflammatory cytokines, and pulmonary function compared with the control group (all P < 0.05). The total clinical efficacy rate of the observation group was significantly higher than that of the control group [96.23% vs. 82.00%; χ 2 = 5.459, P = 0.019; odds ratio (OR) = 5.59, 95% CI: 1.14-27.38; the wide confidence interval, driven by the small number of ineffective cases, indicates that the magnitude of the efficacy advantage should be interpreted with caution]. The total incidence of adverse reactions was comparable between the observation group and the control group (11.32% vs. 10.00%; χ 2 = 0.047, P = 0.828), indicating acceptable safety. The triple combination of ambroxol hydrochloride, montelukast sodium, and azithromycin is associated with improved inflammatory status, enhanced immune function, and better pulmonary function in children with SMPP, with a favorable safety profile. These findings warrant confirmation in prospective, multicenter randomized controlled trials.

PMID 42609515
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PubMedInfection and drug resistance2026-07-22

Association of Ambroxol Hydrochloride and Clenbuterol Hydrochloride Oral Solution with Respiratory Symptom Improvement and Safety in Chinese Children with Pneumonia: A Real-World Propensity Score-Matched Study.

Liang Ying Y, Ling Yesheng Y, Hu Bing B, Zou Yingxue Y et al.

To evaluate the association of ambroxol hydrochloride and clenbuterol hydrochloride oral solution (AHCHOS) with respiratory symptom improvement and safety in Chinese children with pneumonia using real-world data. A propensity score-matched (PSM) cohort study was conducted. Children patients (≤14 years) with a diagnosis of pneumonia between May, 2018 and July, 2019 were considered as the study population. The main outcome of interest was the overall rate of improvement of respiratory symptoms in treatment groups with or without AHCHOS at day 7 using respiratory symptom scores (QS) and Visual Analog Scale (VAS) score of severity of respiratory signs. Secondary end points include medication adherence assessment and safety assessment. A total of 3103 children with a diagnosis of pneumonia were included. After propensity score matching, a sample of 1428 patients was analyzed. AHCHOS use was associated with greater improvement in cough score (p=0.01) and day 7 clinical sign improvement rate (p=0.03) compared with no AHCHOS use. Exploratory subgroup analyses suggested larger differences in children aged 3-6 years and in selected severity strata. Medication adherence assessmentdid not differ significantly between groups at days 4, 7, 14, and 28. Adverse events were similar between groups. In this real-world propensity score-matched analysis, AHCHOS use was associated with improved respiratory symptom outcomes in children with pneumonia and appeared to have an acceptable safety profile. These findings should be interpreted as observational and hypothesis-generating. The study was registered at Chinese Clinical Trial Registry (https://www.chictr.org.cn/, ChiCTR1800015818).

PMID 42483308
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PubMedTranslational pediatrics2026-07-11

Efficacy and safety of inhaled ambroxol hydrochloride solution in Chinese pediatric patients with acute lower respiratory tract infections: a real-world, multicenter, open-label, single-arm study.

Wang Hao H, Zou Yingxue Y, Shang Yunxiao Y, Chen Mingwu M et al.

Although randomized controlled trials (RCTs) have confirmed the mucolytic efficacy of ambroxol hydrochloride solution for inhalation (AHSI) in selected cohorts, their stringent exclusion criteria often omit children with comorbidities and complex presentations encountered in routine practice. Consequently, real-world evidence is needed to evaluate the effectiveness and safety of AHSI in a broader, clinically representative pediatric population with acute lower respiratory tract infections (ALRTIs). This study therefore aimed to assess the real-world effectiveness, safety, and nebulizer compatibility of a 7-day AHSI regimen added to standard care in a large, multicenter cohort of hospitalized pediatric patients with ALRTI. This real-world, multicenter, open-label, single-arm study enrolled hospitalized patients aged ≥6 months with ALRTI (acute bronchitis, bronchiolitis, or pneumonia) and symptom duration <7 days across 62 centers in China (April 2021-April 2022). Key inclusion criteria included a cough score ≥2 (0-4 scale), tenacious sputum, and difficulty expectorating. Major exclusions comprised severe pneumonia, bronchial asthma, interstitial lung disease, significant hepatic or renal dysfunction [alanine aminotransferase (ALT) >1.5× upper limit of normal (ULN), total bilirubin (TBil) or serum creatinine (Scr) > ULN], other severe comorbidities, known hypersensitivity to ambroxol, or recent trial participation. Participants received weight-based doses of nebulized AHSI twice daily for 7 days as add-on to standard care. Follow-up visits occurred at day 4 and day 7 (end of treatment). Primary endpoints were the cough improvement rate (defined by a reduction in cough score) and the overall clinical response rate (investigator-assessed improvement). Secondary endpoints included changes from baseline in cough, throat rales, and pulmonary auscultation scores. Safety assessments comprised monitoring of adverse events (AEs) (coded with MedDRA), vital signs, and laboratory tests (hematology, biochemistry, urinalysis) at baseline and day 7. A total of 2,599 children were enrolled [full analysis set (FAS)]. At baseline, mean age was 3.60±2.50 years, 57.6% were male, and symptom scores were: cough 2.10±0.30, throat sputum 1.65±0.59, lung auscultation 1.44±0.70. In the FAS, the cough improvement rate was 96.73% [95% confidence interval (CI): 96.05-97.41] and the clinical response rate was 94.73% (95% CI: 93.87-95.59). All symptom scores decreased significantly from baseline to day 7 (P<0.001). Drug-related AEs (DRAEs) occurred in 0.39% of patients, predominantly mild-to-moderate rash, transient liver enzyme elevations, and gastrointestinal events; no serious DRAEs were reported. Outcomes were consistent across pneumonia and bronchitis subgroups and across various nebulizer types. This large real-world study demonstrated that a 7-day course of AHSI, added to standard care, was associated with clinically meaningful improvements in respiratory symptoms and a favorable safety profile in children with ALRTI. The consistent effects across disease subtypes and nebulizer devices underscore the practical utility of AHSI in diverse pediatric settings. While the single-arm design limits causal inference, these findings provide robust real-world evidence supporting AHSI as an effective expectorant option. Prospective confirmation through RCTs will further define its role in first-line therapy.

PMID 42433940
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