CLCN1 Antibody (PACO45746)
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주문- SKU:
- PACO45746
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IF
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | CLCN1 Antibody (PACO45746) |
Antibody SKU: | PACO45746 |
Size: | 50ug |
Host Species: | Rabbit |
Tested Applications: | ELISA, IF |
Recommended Dilutions: | ELISA:1:2000-1:10000, IF:1:50-1:200 |
Species Reactivity: | Human |
Immunogen: | Recombinant Human Chloride channel protein 1 protein (634-988AA) |
Form: | Liquid |
Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
Purification Method: | >95%, Protein G purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Immunofluorescent analysis of HepG2 cells using PACO45746 at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). |
Background: | Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport. |
Synonyms: | Chloride channel protein 1 (ClC-1) (Chloride channel protein, skeletal muscle), CLCN1, CLC1 |
UniProt Protein Function: | CLCN1: Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport. Defects in CLCN1 are the cause of Thomsen disease (THD); also known as autosomal dominant myotonia congenita (MCD). THD is characterized by skeletal muscle stiffness (delayed relaxation), due to membrane hyperexcitability. A variant form of Thomsen disease is myotonia levior that is characterized by milder symptoms, later onset and absence of muscle hypo- and hypertrophy. Defects in CLCN1 are the cause of autosomal recessive myotonia congenita (MCR); also known as Becker disease. Belongs to the chloride channel (TC 2.A.49) family. ClC-1/CLCN1 subfamily. |
UniProt Protein Details: | Protein type:Membrane protein, integral; Transporter; Channel, chloride; Membrane protein, multi-pass; Transporter, ion channel Chromosomal Location of Human Ortholog: 7q35 Cellular Component: integral to plasma membrane; plasma membrane; sarcolemma Molecular Function:chloride channel activity; voltage-gated chloride channel activity Biological Process: muscle contraction; transport; action potential propagation; transmembrane transport Disease: Myotonia Congenita, Autosomal Recessive; Myotonia Congenita, Autosomal Dominant |
NCBI Summary: | The CLCN family of voltage-dependent chloride channel genes comprises nine members (CLCN1-7, Ka and Kb) which demonstrate quite diverse functional characteristics while sharing significant sequence homology. The protein encoded by this gene regulates the electric excitability of the skeletal muscle membrane. Mutations in this gene cause two forms of inherited human muscle disorders: recessive generalized myotonia congenita (Becker) and dominant myotonia (Thomsen). Alternative splicing results in multiple transcript variants. [provided by RefSeq, Mar 2012] |
UniProt Code: | P35523 |
NCBI GenInfo Identifier: | 311033468 |
NCBI Gene ID: | 1180 |
NCBI Accession: | P35523.3 |
UniProt Secondary Accession: | P35523,Q2M202, A4D2H5, |
UniProt Related Accession: | P35523 |
Molecular Weight: | 988 |
NCBI Full Name: | Chloride channel protein 1 |
NCBI Synonym Full Names: | chloride channel, voltage-sensitive 1 |
NCBI Official Symbol: | CLCN1 |
NCBI Official Synonym Symbols: | CLC1 |
NCBI Protein Information: | chloride channel protein 1; clC-1; chloride channel 1, skeletal muscle; chloride channel protein, skeletal muscle |
UniProt Protein Name: | Chloride channel protein 1 |
UniProt Synonym Protein Names: | Chloride channel protein, skeletal muscle |
Protein Family: | Chloride channel protein |
UniProt Gene Name: | CLCN1 |
UniProt Entry Name: | CLCN1_HUMAN |