MYH6+Slow Skeletal Myosin Heavy chain Antibody (Rabbit mAb) [E13G18]

Application: Reactivity: Mouse, Rat, Human

Usage Information

Dilution
WB1:500 IHC1:1000 - 1:2000

Application
WB, IHC

Reactivity
Mouse, Rat, Human

Source
Rabbit Monoclonal Antibody

Storage Buffer
PBS, pH 7.2+50% Glycerol+0.05% BSA+0.01% NaN3

Storage (from the date of receipt)
-20°C (avoid freeze-thaw cycles), 2 years

Predicted MW
224 kDa

Datasheet & SDS

Biological Description

Specificity
MYH6+Slow Skeletal Myosin Heavy chain Antibody (Rabbit mAb) [E13G18] detects endogenous levels of total MYH6+Slow Skeletal Myosin Heavy chain protein.

Clone
E13G18

Synonym(s)
MYHCA, MYH6, Myosin-6, Myosin heavy chain 6, MyHC-alpha, MYHCB, MYH7, Myosin-7, Myosin heavy chain 7, Myosin heavy chain slow isoform, MyHC-slow, MyHC-beta

Background
MYH6 encodes the cardiac α-myosin heavy chain, a sarcomeric motor protein of the myosin II family whose head domain hydrolyzes ATP and generates force along actin filaments, while slow skeletal myosin heavy chain isoforms share the same basic myosin architecture but are enriched in oxidative, fatigue-resistant skeletal fibers that support sustained, low-velocity contraction. The heavy chain polypeptide folds into an N-terminal motor domain containing the ATP-binding pocket and actin-interaction interface, followed by a neck region that binds regulatory and essential light chains and a long coiled-coil tail formed by a repetitive heptad pattern that dimerizes to build the thick filament backbone; limited proteolysis yields globular S1 heads and an S2/LMM rod segment, reflecting these functional and structural subdivisions. In atrial myocardium and conduction tissue, α-myosin heavy chain contributes to rapid cross-bridge cycling and efficient atrial contraction, and the MYH6 locus also embeds an intronic microRNA (miR-208a) that regulates expression of other myosin isoforms and conduction-related genes, so MYH6 products influence both mechanical performance and gene networks that shape the electrical phenotype of the atria. MYH6 is a central sarcomeric disease gene for familial atrial septal defects and a strong susceptibility locus for sick sinus syndrome, with rare and common variants in the α-myosin heavy chain altering sinus node function and atrial conduction; a missense variant p.Arg721Trp in the converter domain associates with a markedly increased lifetime risk of sick sinus syndrome and need for pacemaker implantation, consistent with the converter’s role in transmitting conformational changes from the motor domain to the coiled-coil tail during the power stroke. Additional MYH6 mutations, including in-frame deletions within the coiled-coil region that perturbs binding to myosin-binding protein C, impair sarcomere organization and slow conduction in atrial cardiomyocytes, and knockdown of myh6 in zebrafish reduces heart rate in a manner that can be rescued by wild-type but not mutant human α-myosin, indicating that a structurally intact MYH6 heavy chain is required for normal atrial excitation–contraction coupling and sinus node output. Across cardiac and slow skeletal muscle, α- and slow myosin heavy chains act as ATP-driven motors embedded in highly ordered thick filaments, with their head and converter domains setting contraction kinetics and their coiled-coil tails providing filament assembly interfaces, and MYH6 variants that disturb these domains or embedded regulatory microRNAs contribute to structural heart disease, atrial septal malformations, and primary conduction disorders such as sick sinus syndrome.

Background

MYH6 encodes the cardiac α-myosin heavy chain, a sarcomeric motor protein of the myosin II family whose head domain hydrolyzes ATP and generates force along actin filaments, while slow skeletal myosin heavy chain isoforms share the same basic myosin architecture but are enriched in oxidative, fatigue-resistant skeletal fibers that support sustained, low-velocity contraction. The heavy chain polypeptide folds into an N-terminal motor domain containing the ATP-binding pocket and actin-interaction interface, followed by a neck region that binds regulatory and essential light chains and a long coiled-coil tail formed by a repetitive heptad pattern that dimerizes to build the thick filament backbone; limited proteolysis yields globular S1 heads and an S2/LMM rod segment, reflecting these functional and structural subdivisions. In atrial myocardium and conduction tissue, α-myosin heavy chain contributes to rapid cross-bridge cycling and efficient atrial contraction, and the MYH6 locus also embeds an intronic microRNA (miR-208a) that regulates expression of other myosin isoforms and conduction-related genes, so MYH6 products influence both mechanical performance and gene networks that shape the electrical phenotype of the atria. MYH6 is a central sarcomeric disease gene for familial atrial septal defects and a strong susceptibility locus for sick sinus syndrome, with rare and common variants in the α-myosin heavy chain altering sinus node function and atrial conduction; a missense variant p.Arg721Trp in the converter domain associates with a markedly increased lifetime risk of sick sinus syndrome and need for pacemaker implantation, consistent with the converter’s role in transmitting conformational changes from the motor domain to the coiled-coil tail during the power stroke. Additional MYH6 mutations, including in-frame deletions within the coiled-coil region that perturbs binding to myosin-binding protein C, impair sarcomere organization and slow conduction in atrial cardiomyocytes, and knockdown of myh6 in zebrafish reduces heart rate in a manner that can be rescued by wild-type but not mutant human α-myosin, indicating that a structurally intact MYH6 heavy chain is required for normal atrial excitation–contraction coupling and sinus node output. Across cardiac and slow skeletal muscle, α- and slow myosin heavy chains act as ATP-driven motors embedded in highly ordered thick filaments, with their head and converter domains setting contraction kinetics and their coiled-coil tails providing filament assembly interfaces, and MYH6 variants that disturb these domains or embedded regulatory microRNAs contribute to structural heart disease, atrial septal malformations, and primary conduction disorders such as sick sinus syndrome.

References
https://pubmed.ncbi.nlm.nih.gov/19922871/ https://pubmed.ncbi.nlm.nih.gov/21378987/

Protein Size (kDa)	PVDF Transfer Membrane Pore Size (μm)
< 10	0.22
10-20	0.22
20-30	0.45
30-45	0.45
45-70	0.45
80-100	0.45
100-140	0.45
> 140	0.45

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Protein Size (kDa)	Separating Gel Percentage
4-40	20%
12-45	15%
10-70	12.5%
15-100	10%
25-100	8%
60-210	5%