Anti-MYOT 항체 (CAB6439)

Antibody Name:	Anti-MYOT Antibody
Antibody SKU:	CAB6439
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 259-314 of human MYOT (NP_001129412.1).

Application:	WB IHC IF
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:10 - 1:100
Reactivity:	Human, Mouse, Rat
Positive Samples:	293T, HepG2, Mouse skeletal muscle, Mouse heart, Rat skeletal muscle, Rat heart

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 259-314 of human MYOT (NP_001129412.1).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	RPNQ TLPA PKQL RVRP TFSK YLAL NGKG LNVK QAFN PEGE FQRL AAQS GLYE SEEL
Gene ID:	9499
Uniprot:	Q9UBF9
Cellular Location:	Cell membrane, Cytoplasm, Z line, cytoskeleton, myofibril, sarcolemma, sarcomere
Calculated MW:	35kDa/55kDa
Observed MW:	55kDa

Synonyms:

MYOT, LGMD1, LGMD1A, MFM3, TTID, TTOD, myotilin

Background:

This gene encodes a cystoskeletal protein which plays a significant role in the stability of thin filaments during muscle contraction. This protein binds F-actin, crosslinks actin filaments, and prevents latrunculin A-induced filament disassembly. Mutations in this gene have been associated with limb-girdle muscular dystrophy and myofibrillar myopathies. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined.

UniProt Protein Function:	MYOT: Component of a complex of multiple actin cross-linking proteins. Involved in the control of myofibril assembly and stability at the Z lines in muscle cells. Defects in MYOT are the cause of limb-girdle muscular dystrophy type 1A (LGMD1A). LGMD1A is an autosomal dominant degenerative myopathy with onset within a mean age of 28 years. LGMD1A is characterized by progressive skeletal muscle weakness of the hip and shoulder girdles, later progressing to include distal weakness, as well as a distinctive dysarthric pattern of speech. Affected muscle exhibits disorganization and streaming of the Z-line. Defects in MYOT are the cause of myopathy myofibrillar type 3 (MFM3). A neuromuscular disorder characterized by progressive skeletal muscle weakness greater distally than proximally, tight heel cords, hyporeflexia, cardiomyopathy and peripheral neuropathy in some patients. Affected muscle exhibits disorganization and streaming of the Z-line, presence of large hyaline structures, excessive accumulation of myotilin and other ectopically expressed proteins and prominent congophilic deposits. Defects in MYOT are the cause of spheroid body myopathy (SBM). SBM is an autosomal dominant form of myofibrillar myopathy (MFM), characterized by slowly progressing proximal muscle weakness and dysarthric nasal speech. There is no evidence of cardiomyopathy. Muscle biopsy shows spheroid bodies within the type I muscle fibers. Belongs to the myotilin/palladin family. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:	Protein type:Cytoskeletal Chromosomal Location of Human Ortholog: 5q31 Cellular Component: Z disc; sarcolemma; actin cytoskeleton Molecular Function:protein binding; structural constituent of muscle; alpha-actinin binding; actin binding Biological Process: muscle contraction Disease: Myopathy, Spheroid Body; Muscular Dystrophy, Limb-girdle, Type 1a; Myopathy, Myofibrillar, 3
NCBI Summary:	This gene encodes a cystoskeletal protein which plays a significant role in the stability of thin filaments during muscle contraction. This protein binds F-actin, crosslinks actin filaments, and prevents latrunculin A-induced filament disassembly. Mutations in this gene have been associated with limb-girdle muscular dystrophy and myofibrillar myopathies. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined.[provided by RefSeq, Oct 2008]
UniProt Code:	Q9UBF9
NCBI GenInfo Identifier:	311033402
NCBI Gene ID:	9499
NCBI Accession:	Q9UBF9.2
UniProt Secondary Accession:	Q9UBF9,A0A4R6, B4DT79,
UniProt Related Accession:	Q9UBF9
Molecular Weight:	498
NCBI Full Name:	Myotilin
NCBI Synonym Full Names:	myotilin
NCBI Official Symbol:	MYOT
NCBI Official Synonym Symbols:	MFM3; TTID; TTOD; LGMD1; LGMD1A
NCBI Protein Information:	myotilin; 57 kDa cytoskeletal protein; myofibrillar titin-like Ig domains protein; titin immunoglobulin domain protein (myotilin)
UniProt Protein Name:	Myotilin
UniProt Synonym Protein Names:	57 kDa cytoskeletal protein; Myofibrillar titin-like Ig domains protein; Titin immunoglobulin domain protein
Protein Family:	Myotilin
UniProt Gene Name:	MYOT
UniProt Entry Name:	MYOTI_HUMAN

	Western blot analysis of extracts of various cell lines, using MYOT antibody (CAB6439) at 1:1000 dilution._Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution._Lysates/proteins: 25ug per lane._Blocking buffer: 3% nonfat dry milk in TBST._Detection: ECL Enhanced Kit (RM00021)._Exposure time: 90s.
	Immunohistochemistry of paraffin-embedded mouse heart using MYOT antibody (CAB6439) at dilution of 1:100 (40x lens).
	Immunofluorescence analysis of U2OS cells using MYOT antibody (CAB6439). Blue: DAPI for nuclear staining.

Antibodies

MYOT Antibody (PACO10711)

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)