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Recombinant Mouse Anti-MYBPC1 Recombinant Antibody (83B24) (CBMAB-XB0856-YC)

Provided herein is a Mouse Recombinant Antibody against Myosin Binding Protein C, Slow Type. The antibody can be used for immunoassay techniques, such as WB.
See all MYBPC1 antibodies

Summary

Host Animal
Mouse
Specificity
Human, Mouse, Rat
Clone
83B24
Antibody Isotype
IgG1
Application
WB

Basic Information

Immunogen
Crude preparation of human skeletal myosin
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at-20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
myosin binding protein C, slow type
Introduction
MYBPC1 is a member of the myosin-binding protein C family. Myosin-binding protein C family members are myosin-associated proteins found in the cross-bridge-bearing zone (C region) of A bands in striated muscle. The encoded protein is the slow skeletal muscle isoform of myosin-binding protein C and plays an important role in muscle contraction by recruiting muscle-type creatine kinase to myosin filaments. Mutations in this gene are associated with distal arthrogryposis type I.
Entrez Gene ID
Human4604
Mouse109272
Rat362867
UniProt ID
HumanQ00872
MouseQ6P6L5
RatQ63518
Alternative Names
Myosin Binding Protein C, Slow Type; C-Protein, Skeletal Muscle Slow Isoform; Myosin-Binding Protein C, Slow-Type; Slow MyBP-C; MYBPCS; Skeletal Muscle C-Protein; MYBPCC; LCCS4;
Function
Thick filament-associated protein located in the crossbridge region of vertebrate striated muscle a bands. Slow skeletal protein that binds to both myosin and actin (PubMed:31264822, PubMed:31025394).

In vitro, binds to native thin filaments and modifies the activity of actin-activated myosin ATPase. May modulate muscle contraction or may play a more structural role.
Biological Process
Cell adhesion Source: UniProtKB-KW
Muscle contraction Source: Ensembl
Cellular Location
Cytosol
Other locations
Myofibril
Myosin filament
Involvement in disease
Arthrogryposis, distal, 1B (DA1B):
A form of distal arthrogryposis, a disease characterized by congenital joint contractures that mainly involve two or more distal parts of the limbs, in the absence of a primary neurological or muscle disease. Distal arthrogryposis type 1 is characterized largely by camptodactyly and clubfoot. Hypoplasia and/or absence of some interphalangeal creases is common. The shoulders and hips are less frequently affected.
Lethal congenital contracture syndrome 4 (LCCS4):
A form of lethal congenital contracture syndrome, an autosomal recessive disorder characterized by degeneration of anterior horn neurons, extreme skeletal muscle atrophy and congenital non-progressive joint contractures. The contractures can involve the upper or lower limbs and/or the vertebral column, leading to various degrees of flexion or extension limitations evident at birth.
Myopathy, congenital, with tremor (MYOTREM):
An autosomal dominant muscular disorder characterized by muscle weakness, hypotonia associated with high-frequency postural tremor of the limbs, mildly delayed walking, and steppage gait. Additional features include skeletal deformities such as scoliosis, thoracic asymmetry and spinal rigidity. Some patients show mild facial dysmorphic features. Cognitive functions are normal.

Leduc-Pessah, H., Smith, I. C., Kernohan, K. D., Sampaio, M., Melkus, G., Strasser, L., ... & Warman-Chardon, J. (2024). Congenital tremor and myopathy secondary to novel MYBPC1 variant. Journal of the Neurological Sciences, 122864.

Liu, J., Song, J., & Li, C. (2023). MYBPC1 is a key regulator for laryngeal carcinoma formation. Anti-Cancer Drugs, 34(1), 1.

Jiang, S. N., Cao, J. W., Liu, L. Y., Zhou, Y., Shan, G. Y., Fu, Y. H., ... & Yu, Y. C. (2023). Sncg, Mybpc1, and Parm1 Classify subpopulations of VIP-expressing interneurons in layers 2/3 of the somatosensory cortex. Cerebral Cortex, 33(8), 4293-4304.

Zhao, Y., Yan, C., & Ji, K. (2023). Ultrasonography of Muscle Vibration Caused by MYBPC1 Variant. JAMA neurology.

Uneoka, S., Kobayashi, T., Numata-Uematsu, Y., Oikawa, Y., Katata, Y., Okubo, Y., ... & Uematsu, M. (2023). A case series of patients with MYBPC1 gene variants featuring undulating tongue movements as myogenic tremor. Pediatric Neurology.

Hauserman, J. G., Stavusis, J., Joca, H. C., Robinett, J. C., Hanft, L., Vandermeulen, J., ... & Kontrogianni-Konstantopoulos, A. (2021). Sarcomeric deficits underlie MYBPC1-associated myopathy with myogenic tremor. JCI insight, 6(19).

Guo, W. L., Geng, J., Zhao, J. G., Fang, F., Huang, S. G., & Wang, J. (2019). Gene expression profiling reveals upregulated FUT1 and MYBPC1 in children with pancreaticobiliary maljunction. Brazilian Journal of Medical and Biological Research, 52.

Shashi, V., Geist, J., Lee, Y., Yoo, Y., Shin, U., Schoch, K., ... & Marwaha, S. (2019). Heterozygous variants in MYBPC1 are associated with an expanded neuromuscular phenotype beyond arthrogryposis. Human mutation, 40(8), 1115-1126.

Stavusis, J., Lace, B., Schäfer, J., Geist, J., Inashkina, I., Kidere, D., ... & Bönnemann, C. G. (2019). Novel mutations in MYBPC1 are associated with myogenic tremor and mild myopathy. Annals of neurology, 86(1), 129-142.

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For research use only. Not intended for any clinical use.

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