Muscular Dystrophy Related Research Tools
Muscular dystrophy (MD) is an inherited genetic condition that gradually causes the weakening of muscles. The MDs are caused by mutations in a large number of genes with different functions, encoding proteins of the contractile apparatus, structural proteins, enzymes or nuclear proteins. The most commonly encountered forms of the diseases in adults are myotonic dystrophy (MD), facioscapulohumeral muscular dystrophy (FSHD) and the large group of limb-girdle muscular dystrophy (LGMD). The most common childhood form is Duchenne muscular dystrophy (DMD).
Fig.1 Overview of inflammatory mechanism in DMD muscles and related potential drugs. (Miyatake, 2016)
The genomic mutations in MDs exert their pathogenicity at the level of deoxyribonucleic acid (DNA) or ribonucleic acid (RNA). Therefore, the primary molecular targets in MDs are the affected genes and/or their respective downstream targets. Currently, most of the therapeutic strategies are focused on the mutated gene products or their downstream targets. Evidence has shown that the structural linkage between the muscle extracellular matrix and the cytoskeleton is crucial in preventing the progression of MD. The primary mutations of enzymes involved in the protein glycosylation pathways have shown to disrupt the matrix-cell interaction in a certain group of MDs.
Fig.2 Illustration of proteins and potential targets involved in MDs. (Kinter, 2014)
In the past several years, viral and non-viral gene therapy methodologies based on adeno-associated viruses, naked plasmid delivery, antisense oligonucleotides, and oligonucleotide-mediated gene editing have moved from the laboratories to the clinical studies. Creative Biolabs offers comprehensive products as well as high-quality customized services for our clients. We have some of the best products and packages to aid your MDs research.
Summary of the Popular Objects in Muscular Dystrophy
Calpain protease inhibitor | Collagen VI | Creatine |
Dystrophin | Follistatin | Fukutin |
Fukutin-related protein (FKRP) | IGF1 | Integrin α7β1 |
ISS-N1 | Laminin-2 | LARGE |
Leukemia inhibitory factor (LIF) | NFκB | NOX |
P2RX7 purinoceptor | POMT1 | POMT2 |
ROS | ROX | RyR1 |
TNFR | TNFα | TRPV2 |
β-Sarcoglycan | Corticosteroids prednisone | Leukemia inhibitory factor (LIF) |
References
- Miyatake, S.; et al. Anti-inflammatory drugs for Duchenne muscular dystrophy: focus on skeletal muscle-releasing factors. Drug design, development and therapy. 2016, 10: 2745.
- Kinter, J.; Sinnreich, M. Molecular targets to treat muscular dystrophies. Swiss medical weekly. 2014, 144(0708).
Target


Laminin alpha 4/LAMA4 Rabbit Monoclonal Antibody
- Host Species:
- Rabbit
- Species Reactivity:
- Human
- Applications:
- IHC-P
- Conjugation:
- Unconjugated

Neurofilament Rabbit Monoclonal Antibody
- Host Species:
- Rabbit
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; ICC; IF; FCM; IP
- Conjugation:
- Unconjugated

Anti-CAV BETA 2 Monoclonal Antibody
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- IF; WB; IHC; IP
- Conjugation:
- Unconjugated

Mouse monoclonal to BAX
- Host Species:
- Mouse
- Species Reactivity:
- Mouse
- Applications:
- WB; ELISA; IP
- Conjugation:
- Unconjugated

Mouse monoclonal to SSH1
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- WB; ELISA
- Conjugation:
- Unconjugated

- Host Species:
- Rabbit
- Species Reactivity:
- Human
- Applications:
- WB; ELISA; IHC-P; IHC-F; FC; ICC; IF
- Conjugation:
- Unconjugated

Mouse monoclonal to SETD2
- Host Species:
- Mouse
- Species Reactivity:
- Human; Mouse; Rat
- Applications:
- WB
- Conjugation:
- Unconjugated

Mouse monoclonal to GAD1
- Host Species:
- Mouse
- Species Reactivity:
- Human; Monkey; Mouse; Rat
- Applications:
- IF; IHC; IP; WB
- Conjugation:
- Unconjugated

Mouse monoclonal to FABP3
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- ELISA
- Conjugation:
- Unconjugated

Mouse monoclonal to NEFL
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- ELISA
- Conjugation:
- Unconjugated

Mouse monoclonal to GFAP
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- ELISA
- Conjugation:
- Unconjugated

Mouse monoclonal to GFAP
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- ELISA
- Conjugation:
- Unconjugated

Mouse monoclonal to UCHL1
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- ELISA
- Conjugation:
- Unconjugated

Mouse monoclonal to UCHL1
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- ELISA
- Conjugation:
- Unconjugated

Mouse Monoclonal to IL1A
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- IF; WB
- Conjugation:
- Unconjugated

Mouse Monoclonal to APOE
- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- IHC; WB
- Conjugation:
- Unconjugated; APC; PE; HRP; Biotin; FITC

- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- WB; IP; ELISA

- Host Species:
- Mouse
- Species Reactivity:
- Human; Bovine
- Applications:
- WB; IP; IF

- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; IHC-P; ELISA

- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; IHC-P; ELISA

- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; ELISA

- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- WB; IP; IF; IHC-P; ELISA

- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; ELISA

- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; ELISA

- Host Species:
- Mouse
- Species Reactivity:
- Human; Feline
- Applications:
- WB; IP; IF; IHC-P; FC

- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; IHC-P; ELISA

- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; ELISA

- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; IHC-P

- Host Species:
- Mouse
- Species Reactivity:
- Mouse; Rat; Human
- Applications:
- WB; IP; IF; ELISA

- Host Species:
- Mouse
- Species Reactivity:
- Human
- Applications:
- WB; IP; IF; ELISA
- iNeuMab™ Anti-CD32b Antibody (NRP-0422-P1803) (Cat#: NRP-0422-P1803)
- iNeuMab™ Rabbit Anti-LRRK2 Monoclonal Antibody (CBP1887) (Cat#: NAB-08-PZ735)
- iNeuMab™ Anti-Tau Antibody (NRP-0422-P1683) (Cat#: NRP-0422-P1683)
- iNeuMab™ Anti-pTau Antibody (NRP-0422-P1719) (Cat#: NRP-0422-P1719)
- iNeuMab™ Anti-F-Spondin/SPON1 Antibody, Clone 3F4 (Cat#: NRZP-0822-ZP4740)
- iNeuMab™ Anti-Tau Antibody (NRP-0422-P1686) (Cat#: NRP-0422-P1686)
- iNeuMab™ Anti-TNFα BBB Shuttle Antibody (NRZP-1022-ZP4105) (Cat#: NRZP-1022-ZP4105)
- iNeuMab™ Anti-SEZ6 Antibody (NRP-0422-P517) (Cat#: NRP-0422-P517)
- iNeuMab™ Anti-ApoC3 BBB Shuttle Antibody (NRZP-1022-ZP3503) (Cat#: NRZP-1022-ZP3503)
- iNeuMab™ Anti-ApoC3 BBB Shuttle Antibody (NRZP-1022-ZP3505) (Cat#: NRZP-1022-ZP3505)
- Mouse Microglia from C57BL/6 (Cat#: NCL-21P6-082)
- Mouse Retinal Ganglion Cell Line RGC-5 (Cat#: NCL2110P154)
- Human Brain Vascular Adventitial Fibroblasts (Cat#: NCL-21P6-014)
- Mouse Glioma Cell Line GL261-GFP (Cat#: NCL-2108P04)
- iNeu™ Human Motor Neurons (Cat#: NCL-2103-P71)
- Mouse Microglia N9 (Cat#: NCL2110P073)
- Human Neurons Isolated from Cortex (Cat#: NCL-21P6-023)
- Rat Retinal Muller Cell Line, Immortalized (Cat#: NCL-21P6-192)
- Human Blood Brain Barrier Model (Cat#: NCL-2103-P187)
- Green Fluorescent Alpha-synuclein Cell Line (Cat#: NCL2110P209)
- Human GFAP ELISA Kit [Colorimetric] (Cat#: NPP2011ZP383)
- Beta Amyloid (1-40), Aggregation Kit (Cat#: NRZP-0323-ZP199)
- Alpha-Synuclein Aggregation Assay Kit (Cat#: NRZP-1122-ZP37)
- Beta Amyloid (1-42), Aggregation Kit (Cat#: NRZP-0323-ZP200)
- Human Tau Aggregation Kit (Cat#: NRP-0322-P2173)
- Amyloid beta 1-42 Kit (Cat#: NRP-0322-P2170)
- Alpha Synuclein Aggregation Kit (Cat#: NRZP-1122-ZP15)
- Human Poly ADP ribose polymerase,PARP Assay Kit (Cat#: NRZP-1122-ZP62)
- AAV2 Full Capsids, Reference Standards (Cat#: NTC2101070CR)
- AAV2/9-hEF1a-fDIO-eNpHR 3.0-mCherry-WPRE-pA (Cat#: NTA-2012-ZP78)
- VSV-eGFP (Cat#: NTA-2011-ZP20)
- Dextran, NHS Activated (Cat#: NRZP-0722-ZP124)
- Human superoxide dismutase 3, extracellular (SOD3) (NM_003102) ORF clone, Untagged (Cat#: NEP-0521-R0808)
- Human superoxide dismutase 1, soluble (SOD1) (NM_000454) ORF clone, TurboGFP Tagged (Cat#: NEP-0521-R0748)
- Mouse Parkinson disease (autosomal recessive, early onset) 7 (Park7) (NM_020569) clone, Untagged (Cat#: NEP-0621-R0133)
- Tau Antisense Oligonucleotide (IONIS-MAPTRx) (Cat#: NV-2106-P29)
- App Rat amyloid beta (A4) precursor protein (App)(NM_019288) ORF clone, Untagged (Cat#: NEP-0421-R0053)
- Lenti of Human TAR DNA binding protein (TARDBP) (NM_007375) ORF clone, mGFP Tagged (Cat#: NEP-0521-R0832)
- Human huntingtin-associated protein 1 (HAP1) transcript variant 2 (NM_177977) ORF clone, Myc-DDK Tagged (Cat#: NEP-0521-R0676)
- Human presenilin 1 (PSEN1), transcript variant 2 (NM_007318) ORF clone, TurboGFP Tagged (Cat#: NEP-0421-R0140)
- Human apolipoprotein E (APOE) (NM_000041) ORF clone, Untagged (Cat#: NEP-0421-R0232)
- Human huntingtin (HTT) (NM_002111) ORF clone, Myc-DDK Tagged (Cat#: NEP-0521-R0497)
- NeuroBiologics™ Rat Cerebrospinal Fluid (Cat#: NRZP-0822-ZP496)
- NeuroBiologics™ Mouse Cerebrospinal Fluid (Cat#: NRZP-0822-ZP497)
- NeuroBiologics™ Monkey Cerebrospinal Fluid (Cat#: NRZP-0822-ZP495)
- NeuroBiologics™ Human Cerebrospinal Fluid (Cat#: NRZP-0822-ZP491)
- NeuroBiologics™ Pig Cerebrospinal Fluid (Cat#: NRZP-0822-ZP498)
- NeuroPro™ Anti-IDS BBB Shuttle Protein (Cat#: NRZP-0423-ZP503)
- NeuroPro™ Anti-GDNF BBB Shuttle Protein (Cat#: NRZP-0423-ZP509)
- NeuroPro™ Anti-PON1 BBB Shuttle Protein (Cat#: NRZP-0423-ZP507)
- NeuroPro™ Anti-Erythropoietin BBB Shuttle Protein (Cat#: NRZP-0423-ZP499)
- NeuroPro™ Anti-NAGLU BBB Shuttle Protein (Cat#: NRZP-0423-ZP506)
- NeuroPro™ Anti-EPO BBB Shuttle Protein (Cat#: NRZP-0423-ZP508)
- NeuroPro™ Anti-TNFR BBB Shuttle Protein (Cat#: NRZP-0423-ZP510)
- NeuroPro™ Anti-SGSH BBB Shuttle Protein (Cat#: NRZP-0423-ZP505)
- NeuroPro™ Anti-GDNF BBB Shuttle Protein (Cat#: NRZP-0423-ZP500)
- NeuroPro™ Anti-idursulfase BBB Shuttle Protein (Cat#: NRZP-0423-ZP497)