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).

For Research Use Only. Not For Clinical Use.

Target

PGP9.5

Nogo

RBFOX1

GATA3

GAD1

versican

CAV BETA 2

Neurofilament

GALP

ENTPD2

SSH1

RUNX1

LRRK2

GOSR1

Amyloid Fibril

CDK1

SOX2

SORT1

M1 Muscarinic Receptor

FABP3

HTR3C

4E-BP1

SETD2

GRID1

GEMIN4

Stathmin 1

GFAP

NEUROG2

DCAMKL1

RUNX2

Neurofilament L

FE65

Related Products

NeuroMab™ Rabbit Anti-LAMA4 Monoclonal Antibody (CBP7290) [CAT#: NAB-0720-Z9942]

Laminin alpha 4/LAMA4 Rabbit Monoclonal Antibody

Host Species:: Rabbit

Species Reactivity:: Human

Applications:: IHC-P

Conjugation:: Unconjugated; APC; PE; HRP; Biotin; FITC; Alexa Fluor 488; Alexa Fluor 700; Alexa Fluor 647; Alexa Fluor 750; Alexa Fluor 594; Alexa Fluor 350; Alexa Fluor 3551

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