Advances in Mitochondrial Metabolism in Neurological Diseases
Creative Biolabs describes recent research advances in mitochondrial metabolism in several major neurological disorders and offers the following related services to help accelerate the progress of your program.
Our Services | Descriptions |
Neuronal Mitochondrial Activity Assay | Creative Biolabs has extensive experience in mitochondrial activity testing, and we have successfully developed a variety of assays for mitochondrial activity assessment. The combination of our fluorescence imaging technology, electrophysiology, and brain slice preparation provides an attractive tool for studying mitochondrial function during neuronal activity. |
Neuronal Mitochondrial Membrane Potential Assay | MMP assays that rely on fluorescent probes are useful for probing mitochondrial health, localization, and abundance, as well as screening and monitoring the effects of some pharmaceutical agents. Creative Biolabs provides diversified MMP testing services, especially JC-1 testing. |
Mitochondrial Stress Assay | A variety of methods and assays for mitochondrial stress detection are available at Creative Biolabs. Fluorescent dye-based assays are a commonly used method to measure mitochondrial stress. |
Mitochondrial Metabolism in Alzheimer's Disease (AD)
AD is a common neurodegenerative disorder characterized by progressive cognitive decline and neuronal loss. Mitochondrial dysfunction has been found to be one of the important pathological features of AD.
-
Changes in mitochondrial energy metabolism - Studies have shown that in neurons of AD patients, mitochondrial respiratory chain complex activity is significantly reduced and ATP synthesis is decreased.
- Inadequate mitochondrial energy production leads to impaired neuronal energy metabolism, which in turn affects neuronal survival and function.
- An imbalance in Ca²⁺ homeostasis within mitochondria is also thought to be closely related to the development of AD.
-
Increased oxidative stress - Studies have shown significantly elevated levels of oxidative stress in the brain tissue of AD patients.
- Excessive reactive oxygen species (ROS) damage the mitochondrial membrane and its DNA, causing further deterioration of mitochondrial function.
- Mitochondrial damage is also thought to be one of the factors triggering the abnormal accumulation of β-amyloid, which further contributes to the progression of AD.
- Role of mitophagy - Recent studies have concluded that mitophagy plays an important role in AD. Mitochondrial autophagy maintains the healthy state of intracellular mitochondria by removing damaged mitochondria. Accordingly, dysfunction of mitochondrial autophagy is thought to be closely associated with AD-related neurodegenerative changes.
For mitochondrial metabolism related research, we also offer a range of research tools, including but not limited to the following:
Cat. No | Product Name | Product Category | Applications |
NCL2110P234 | Parkin Mitochondrial Recruitment Assay Cell Line [Parkinson's Disease (PD) Model] | Cell | Cell Culture |
NRYF-0124-HZ1161 | NeuroBiologics™ Normal Whole Brain Mitochondrial Protein Lysates of Human | Biospecimens | WB; IP; SDS-PAGE; Proteomics |
NRYF-0124-HZ1162 | NeuroBiologics™ Normal Whole Brain Mitochondrial Protein Lysates of CD1 (ICR) Mouse | Biospecimens | WB; IP; SDS-PAGE; Proteomics |
NRYF-0124-HZ1163 | NeuroBiologics™ Normal Whole Brain Mitochondrial Protein Lysates of C57BL/6 Mouse | Biospecimens | WB; IP; SDS-PAGE; Proteomics |
NRYF-0124-HZ1164 | NeuroBiologics™ Normal Whole Brain Mitochondrial Protein Lysates of BALB/c Mouse | Biospecimens | WB; IP; SDS-PAGE; Proteomics |
NRYF-0124-HZ1165 | NeuroBiologics™ Normal Whole Brain Mitochondrial Protein Lysates of SD Rat | Biospecimens | WB; IP; SDS-PAGE; Proteomics |
NRYF-0124-HZ1166 | NeuroBiologics™ Normal Whole Brain Mitochondrial Protein Lysates of WS Rat | Biospecimens | WB; IP; SDS-PAGE; Proteomics |
NAB-0720-Z5975 | Rabbit Anti-Mitochondrial Ferritin Monoclonal Antibody (CBP3202) | Antibody | WB |
NAB-0720-Z1007 | NeuroMab™ Rabbit Anti-SUCLA2 Monoclonal Antibody (CBP322) | Antibody | ICC; IF; FC; IHC-P; WB |
NAB-0720-Z1581 | Mouse Anti-OPA1 Monoclonal Antibody (CBP391) | Antibody | WB; FC |
NAB-0720-Z2059 | NeuroMab™ Rabbit Anti-SLC25A12 Monoclonal Antibody (CBP503) | Antibody | WB; IP |
NAB-0720-Z2229 | NeuroMab™ Rabbit Anti-DMGDH Monoclonal Antibody (CBP572) | Antibody | WB; IHC-P |
NAB-08-PZ1201 | NeuroMab™ Rabbit Anti-SOD2 (CBP1702) | Antibody | WB; IHC-P; DB |
Mitochondrial Metabolism in Parkinson's Disease (PD)
PD is a neurological disorder characterized by movement disorders in early childhood, which are mainly characterized by tremor, rigidity, and bradykinesia. Studies have shown that mitochondrial metabolic disorders are closely related to the development of PD.
- Mitochondrial dysfunction - Patients with PD have impaired mitochondrial function in dopaminergic neurons in the brain, as evidenced by decreased mitochondrial ATP synthesis and decreased activity of the respiratory chain complex. Mitochondrial dysfunction leads to insufficient energy production, resulting in decreased neuronal resistance to oxidative stress and ultimately cell death.
- Transcription factors and mitochondrial metabolism - The transcription factors PINK1 and Parkin also play an important role in the pathogenesis of PD. These factors are closely associated with mitochondrial autophagy and are involved in mitochondrial quality control.
- Mitochondria and the inflammatory response - Chronic inflammatory responses occur in the brain of PD patients, and recent studies have found that mitochondria release molecules that activate microglia and further exacerbate neuroinflammation.
Mitochondrial Metabolism in Huntington's Disease (HD)
HD is an inherited neurodegenerative disease characterized by the pathology of death of specific neurons, especially striatal neurons, leading to motor, emotional and cognitive dysfunction.
- Alterations in mitochondrial metabolism - Decreased mitochondrial membrane potential and dysregulation of mitochondrial dynamic homeostasis have been observed in both HD patients and animal models, leading to mitochondrial dysfunction. These abnormalities lead to decreased efficiency of oxidative phosphorylation, energy deficiency and increased apoptosis.
- SIRT1 and mitochondrial protection - The SIRT1 protein has been found to play a protective role in the pathological process of HD. SIRT1 can counteract the metabolic disturbances that occur in HD by promoting mitochondrial biogenesis and improved function through deacetylation.
- Mitochondria and neuroinflammation - Studies have also revealed that in HD, mitochondria-related signaling pathways regulate neuroinflammatory responses that influence disease progression.
Mitochondrial Metabolism in Multiple Sclerosis (MS)
MS is an autoimmune disease characterized by chronic inflammation and demyelination damage in the central nervous system. The role of mitochondrial metabolism in this disease has received equal attention.
Fig. 1 Mitochondrial dysfunction associated with multiple sclerosis.1
- Disturbances in mitochondrial energy metabolism - Mitochondrial function is impaired in glial cells and neurons of MS patients, as evidenced by reduced efficiency of ATP production and oxidative phosphorylation. This metabolic impairment may accelerate neuronal cell death and myelin damage.
- Mitochondria and oxidative stress - Levels of oxidative stress are significantly elevated in the nervous system of MS patients, and mitochondria are one of the major sources of ROS production. Inhibition of mitochondria-generated ROS is thought to attenuate pathological damage in MS.
Abnormalities in mitochondrial metabolism in a variety of neurological disorders are closely linked to disease pathogenesis. With in-depth studies of mitochondrial function, mitochondria-based intervention strategies may be developed in the future to improve the course of these neurological diseases. By repairing mitochondrial function and improving cellular energy metabolism, it may have a positive impact on the treatment of neurological diseases.
Reference
- Barcelos, Isabella Peixoto de, Regina M. Troxell, and Jennifer S. Graves. "Mitochondrial dysfunction and multiple sclerosis." Biology 8.2 (2019): 37. Distributed under Open Access license CC BY 4.0, without modification.
- NeuroMab™ Anti-Integrin αvβ8 BBB Shuttle Antibody(NRZP-1222-ZP1218) (Cat#: NRZP-1222-ZP1218)
- NeuroMab™ Anti-Amyloid Beta 1-15 Antibody(NRP-0422-P867) (Cat#: NRP-0422-P867)
- NeuroMab™ Rabbit Anti-LRRK2 Monoclonal Antibody (CBP1887) (Cat#: NAB-08-PZ735)
- NeuroMab™ Anti-Tau Antibody(NRP-0422-P2275) (Cat#: NRP-0422-P2275)
- NeuroMab™ Anti-TREM2 BBB Shuttle Antibody(NRZP-1022-ZP4114) (Cat#: NRZP-1022-ZP4114)
- NeuroMab™ Anti-ApoC3 BBB Shuttle Antibody(NRZP-1022-ZP3505) (Cat#: NRZP-1022-ZP3505)
- NeuroMab™ Anti-FGFR1 Antibody(NRP-0422-P1244) (Cat#: NRP-0422-P1244)
- NeuroMab™ Anti-GD2 Antibody(NRZP-1222-ZP767) (Cat#: NRZP-1222-ZP767)
- NeuroMab™ Anti-Alpha Synuclein BBB Shuttle Antibody(NRZP-1022-ZP4050) (Cat#: NRZP-1022-ZP4050)
- NeuroMab™ Rabbit Anti-Alpha-synuclein (CBP1631) (Cat#: NAB-08-PZ079)
- Human Blood Brain Barrier Model (Cat#: NCL-2103-P187)
- Human Glial (Oligodendrocytic) Hybrid Cell Line (MO3.13) (Cat#: NCL-2108P34)
- C57 Brain Cortex Neurons [Mouse] (Cat#: NCC20-9PZ48)
- Mouse Retinal Ganglion Cells (Cat#: NCL2110P145)
- Rat Microglia Cell Line HAPI, Immortalized (Cat#: NCL2110P015)
- Mouse Glioma Cell Line GL261 (Cat#: NCL-2108P28)
- Green Fluorescent BACE1 Cell Lines (Cat#: NCL2110P214)
- Human Brain Vascular Pericytes (Cat#: NCL-21P6-015)
- Mouse Microglia N9 (Cat#: NCL2110P073)
- iNeu™ Human Motor Neurons (Cat#: NCL-2103-P71)
- Beta Amyloid (1-40), Aggregation Kit (Cat#: NRZP-0323-ZP199)
- Beta Amyloid (1-42), Aggregation Kit (Cat#: NRZP-0323-ZP200)
- Amyloid beta 1-42 Kit (Cat#: NRP-0322-P2170)
- Alpha-Synuclein Aggregation Assay Kit (Cat#: NRZP-1122-ZP37)
- Human GFAP ELISA Kit [Colorimetric] (Cat#: NPP2011ZP383)
- Human Tau Aggregation Kit (Cat#: NRP-0322-P2173)
- Human Poly ADP ribose polymerase,PARP Assay Kit (Cat#: NRZP-1122-ZP62)
- Alpha Synuclein Aggregation Kit (Cat#: NRZP-1122-ZP15)
- AAV-EF1a-mCherry-flex-dtA (Cat#: NRZP-0622-ZP616)
- pAAV-syn-jGCaMP8f-WPRE (Cat#: NTA-2106-P061)
- AAV2/2Retro-CAG-DIO-EGFP-2A-TetTox-pA [Neural Tracing] (Cat#: NTA-2012-ZP303)
- pAAV-syn-FLEX-jGCaMP8f-WPRE (Cat#: NTA-2106-P064)
- VSV-eGFP (Cat#: NTA-2011-ZP20)
- AAV2/9-hEF1a-fDIO-eNpHR 3.0-mCherry-WPRE-pA (Cat#: NTA-2012-ZP78)
- Dextran-FITC (Cat#: NTA-2011-ZP110)
- pAAV-syn-jGCaMP8s-WPRE (Cat#: NTA-2106-P063)
- Dextran, Cy5 Labeled, 2000 kDa (Cat#: NRZP-0722-ZP22)
- pAAV-syn-FLEX-jGCaMP8m-WPRE (Cat#: NTA-2106-P065)
- ABCA1 Antisense Oligonucleotide (NV-2106-P27) (Cat#: NV-2106-P27)
- Tau Antisense Oligonucleotide (IONIS-MAPTRx) (Cat#: NV-2106-P29)
- Mouse Parkinson disease (autosomal recessive, early onset) 7 (Park7) (NM_020569) clone, Untagged (Cat#: NEP-0621-R0133)
- Rat Parkinson disease (autosomal recessive, juvenile) 2, parkin (Park2) (NM_020093) ORF clone/lentiviral particle, Myc-DDK Tagged (Cat#: NEP-0621-R0041)
- Lenti of Mouse synuclein, alpha (Snca) transcript variant (NM_001042451) ORF clone, mGFP Tagged (Cat#: NEP-0521-R0864)
- Human presenilin 1 (PSEN1), transcript variant 2 (NM_007318) ORF clone, TurboGFP Tagged (Cat#: NEP-0421-R0140)
- Human huntingtin-associated protein 1 (HAP1) transcript variant 2 (NM_177977) ORF clone, Myc-DDK Tagged (Cat#: NEP-0521-R0676)
- Human superoxide dismutase 3, extracellular (SOD3) (NM_003102) ORF clone, Untagged (Cat#: NEP-0521-R0808)
- Lenti of Human TAR DNA binding protein (TARDBP) (NM_007375) ORF clone, mGFP Tagged (Cat#: NEP-0521-R0832)
- App Rat amyloid beta (A4) precursor protein (App)(NM_019288) ORF clone, Untagged (Cat#: NEP-0421-R0053)
- NeuroBiologics™ Pig Cerebrospinal Fluid (Cat#: NRZP-0822-ZP498)
- NeuroBiologics™ Monkey Cerebrospinal Fluid (Cat#: NRZP-0822-ZP495)
- NeuroBiologics™ Rat Cerebrospinal Fluid (Cat#: NRZP-0822-ZP496)
- NeuroBiologics™ Human Cerebrospinal Fluid (Cat#: NRZP-0822-ZP491)
- NeuroBiologics™ Mouse Cerebrospinal Fluid (Cat#: NRZP-0822-ZP497)
- NeuroPro™ Anti-TNFR BBB Shuttle Protein (Cat#: NRZP-0423-ZP510)
- NeuroPro™ Anti-TNFR BBB Shuttle Protein (Cat#: NRZP-0423-ZP501)
- NeuroPro™ Anti-ASA BBB Shuttle Protein (Cat#: NRZP-0423-ZP504)
- NeuroPro™ Anti-IDS BBB Shuttle Protein (Cat#: NRZP-0423-ZP503)
- NeuroPro™ Anti-IDUA BBB Shuttle Protein (Cat#: NRZP-0423-ZP502)
- NeuroPro™ Anti-SGSH BBB Shuttle Protein (Cat#: NRZP-0423-ZP505)
- NeuroPro™ Anti-GDNF BBB Shuttle Protein (Cat#: NRZP-0423-ZP509)
- NeuroPro™ Anti-NAGLU BBB Shuttle Protein (Cat#: NRZP-0423-ZP506)
- NeuroPro™ Anti-GDNF BBB Shuttle Protein (Cat#: NRZP-0423-ZP500)
- NeuroPro™ Anti-Erythropoietin BBB Shuttle Protein (Cat#: NRZP-0423-ZP499)