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ABCA1 Antisense Oligonucleotide (AK311445) (Cat#: NV-2106-P27)
Human huntingtin-associated protein 1 (HAP1) transcript variant 2 (NM_177977) ORF clone, Myc-DDK Tagged (Cat#: NEP-0521-R0676)
App Rat amyloid beta (A4) precursor protein (App)(NM_019288) ORF clone, Untagged (Cat#: NEP-0421-R0053)
Lenti of Mouse synuclein, alpha (Snca) transcript variant (NM_001042451) ORF clone, mGFP Tagged (Cat#: NEP-0521-R0864)
Mouse SOD1 shRNA Silencing Adenovirus (Cat#: NV-2106-P14)
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)
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)

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Cell Assay Reagents

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

Myelin Basic Protein

FABP3

CPLX3

RUNX1

ENTPD2

CARD9

SSH1

Stathmin 1

NEUROG2

GFAP

NrCAM

HTR3C

GATA3

Laminin alpha 4/LAMA4

BAX

GEMIN4

RBFOX1

CD3

Cathepsin D

RUNX2

SORT1

GRID1

RUNX3

Amyloid Fibril

Apo E

GALP

IL-6

Neurofilament L

NPY2

LRRK2

Neurabin 1

SOX2

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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