Anti-FLNC Antibody (CAB13018)
- SKU:
- CAB13018
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
抗体名: | Anti-FLNC Antibody |
抗体コード: | CAB13018 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IF |
反応性: | Human, Mouse |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 2160-2340 of human FLNC (NP_001449.3). |
申し込み: | WB IF |
推奨希釈: | WB 1:500 - 1:2000 IF 1:50 - 1:200 |
反応性: | Human, Mouse |
ポジティブサンプル: | A-549, LO2, NIH/3T3 |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 2160-2340 of human FLNC (NP_001449.3). |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | IPGN WFQM VSAQ ERLT RTFT RSSH TYTR TERT EISK TRGG ETKR EVRV EEST QVGG DPFP AVFG DFLG RERL GSFG SITR QQEG EASS QDMT AQVT SPSG KVEA AEIV EGED SAYS VRFV PQEM GPHT VAVK YRGQ HVPG SPFQ FTVG PLGE GGAH KVRA GGTG LERG VAGV PAEF SIWT R |
遺伝子ID: | 2318 |
Uniprot: | Q14315 |
セルラーロケーション: | Cytoplasm, Membrane, Peripheral membrane protein, Z line, cytoskeleton, myofibril, sarcomere |
計算された分子量: | 287kDa/291kDa |
観察された分子量: | 302kDa |
同義語: | FLNC, ABP-280, ABP280A, ABPA, ABPL, CMH26, FLN2, MFM5, MPD4, RCM5, filamin-C |
バックグラウンド: | This gene encodes one of three related filamin genes, specifically gamma filamin. These filamin proteins crosslink actin filaments into orthogonal networks in cortical cytoplasm and participate in the anchoring of membrane proteins for the actin cytoskeleton. Three functional domains exist in filamin: an N-terminal filamentous actin-binding domain, a C-terminal self-association domain, and a membrane glycoprotein-binding domain. Two transcript variants encoding different isoforms have been found for this gene. |
UniProt Protein Function: | FLNC: Muscle-specific filamin, which plays a central role in muscle cells, probably by functioning as a large actin-cross- linking protein. May be involved in reorganizing the actin cytoskeleton in response to signaling events, and may also display structural functions at the Z lines in muscle cells. Critical for normal myogenesis and for maintaining the structural integrity of the muscle fibers. Defects in FLNC are the cause of myopathy myofibrillar type 5 (MFM5). A neuromuscular disorder, usually with an adult onset, characterized by focal myofibrillar destruction and pathological cytoplasmic protein aggregations, and clinical features of a limb-girdle myopathy. Defects in FLNC are the cause of myopathy distal type 4 (MPD4). MPD4 is a slowly progressive muscular disorder characterized by distal muscle weakness and atrophy affecting the upper and lower limbs. Onset occurs around the third to fourth decades of life, and patients remain ambulatory even after long disease duration. Muscle biopsy shows non-specific changes with no evidence of rods, necrosis, or inflammation. Belongs to the filamin family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Motility/polarity/chemotaxis; Cytoskeletal Chromosomal Location of Human Ortholog: 7q32-q35 Cellular Component: costamere; focal adhesion; cytoskeleton; cytoplasm; plasma membrane; sarcoplasm; Z disc; cytosol; sarcolemma Molecular Function:actin filament binding; protein binding; cytoskeletal protein binding; ankyrin binding Biological Process: muscle fiber development Disease: Myopathy, Myofibrillar, 5; Myopathy, Distal, 4 |
NCBI Summary: | This gene encodes one of three related filamin genes, specifically gamma filamin. These filamin proteins crosslink actin filaments into orthogonal networks in cortical cytoplasm and participate in the anchoring of membrane proteins for the actin cytoskeleton. Three functional domains exist in filamin: an N-terminal filamentous actin-binding domain, a C-terminal self-association domain, and a membrane glycoprotein-binding domain. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008] |
UniProt Code: | Q14315 |
NCBI GenInfo Identifier: | 254763419 |
NCBI Gene ID: | 2318 |
NCBI Accession: | Q14315.3 |
UniProt Secondary Accession: | Q14315,O95303, Q07985, Q9NS12, Q9NYE5, Q9UMR8, Q9Y503 B2ZZ88, |
UniProt Related Accession: | Q14315 |
Molecular Weight: | 291,022 Da |
NCBI Full Name: | Filamin-C |
NCBI Synonym Full Names: | filamin C, gamma |
NCBI Official Symbol: | FLNC |
NCBI Official Synonym Symbols: | ABPA; ABPL; FLN2; MFM5; MPD4; ABP-280; ABP280A |
NCBI Protein Information: | filamin-C; filamin-2; ABP-280-like protein; ABP-L, gamma filamin; actin binding protein 280 |
UniProt Protein Name: | Filamin-C |
UniProt Synonym Protein Names: | ABP-280-like protein; ABP-L; Actin-binding-like protein; Filamin-2; Gamma-filamin |
Protein Family: | Filamin |
UniProt Gene Name: | FLNC |
UniProt Entry Name: | FLNC_HUMAN |
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