C1116
Anti-Chloride Channel CLC-5 (Clcn5) antibody produced in rabbit
affinity isolated antibody, lyophilized powder
Sinônimo(s):
Anti-CLC5, Anti-CLCK2, Anti-ClC-5, Anti-DENT1, Anti-DENTS, Anti-NPHL1, Anti-NPHL2, Anti-XLRH, Anti-XRN
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About This Item
Produtos recomendados
fonte biológica
rabbit
Nível de qualidade
conjugado
unconjugated
forma do anticorpo
affinity isolated antibody
tipo de produto de anticorpo
primary antibodies
clone
polyclonal
Formulário
lyophilized powder
reatividade de espécies
human, mouse, rat
técnica(s)
western blot: 1:200 using rat kidney membranes
nº de adesão UniProt
Condições de expedição
dry ice
temperatura de armazenamento
−20°C
modificação pós-traducional do alvo
unmodified
Informações sobre genes
human ... CLCN5(1184)
mouse ... Clcn5(12728)
rat ... Clcn5(25749)
Imunogênio
peptide corresponding to amino acid residues 401-415 of rat CLC5. Mouse sequence is identical; human sequence is 14/15 residues identical.
Aplicação
Anti-Chloride Channel CLC-5 (Clcn5) antibody produced in rabbit is suitable for western blotting at a dilution of 1:200 using rat kidney membranes.
Ações bioquímicas/fisiológicas
H(+)/Cl(-) exchange transporter 5 is a protein encoded by the CLCN5 gene in humans. It encodes a member of the CLC gene family of chloride ion channels and ion transporters. CLCN5 is highly expressed in endosomes of proximal tubule cells and is essential for endocytosis. Mutations in CLCN5 causes Dent′s disease leading to renal failure. It is also involved in low-molecular-weight proteinuria, hypercalciuria, nephrolithiasis and renal failure. Majority of these disease-causing mutations in ClC-5 are misprocessed and retained in the ER (endoplasmic reticulum) and may alter intramolecular interactions within the full-length ClC-5 protein. CLC-5 plays a crucial role in the process of endocytosis in the proximal tubule of the kidney and mutations that alter protein function are the cause of Dent′s disease. It may act as an electrically shunting Cl- channel in early endosomes, facilitating intraluminal acidification.
forma física
Lyophilized from phosphate buffered saline containing, pH 7.4, 1% BSA and 0.05% sodium azide
Exoneração de responsabilidade
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Código de classe de armazenamento
11 - Combustible Solids
Classe de risco de água (WGK)
WGK 2
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S Waldegger et al.
Journal of the American Society of Nephrology : JASN, 11(7), 1331-1339 (2000-06-23)
Chloride channels are involved in a multitude of physiologic processes ranging from basal cellular functions such as cell volume regulation and acidification of intracellular vesicles to more specialized mechanisms such as vectorial transepithelial transport and regulation of cellular excitability. This
Thomas J Jentsch et al.
Physiological reviews, 82(2), 503-568 (2002-03-28)
Cl- channels reside both in the plasma membrane and in intracellular organelles. Their functions range from ion homeostasis to cell volume regulation, transepithelial transport, and regulation of electrical excitability. Their physiological roles are impressively illustrated by various inherited diseases and
Ashish K Solanki et al.
Kidney international reports, 3(6), 1443-1453 (2018-11-15)
Tubular dysfunction is characteristic of Dent's disease; however, focal segmental glomerulosclerosis (FSGS) can also be present. Glomerulosclerosis could be secondary to tubular injury, but it remains uncertain whether the CLCN5 gene, which encodes an endosomal chloride and/or hydrogen exchanger, plays
Miriam F Figueira et al.
Physiological reports, 5(13) (2017-07-06)
Diabetic nephropathy (DN) occurs in around 40% of those with diabetes. Proteinuria is the main characteristic of DN and develops as a result of increased permeability of the glomerulus capillary wall and/or decreased proximal tubule endocytosis. The goal of this
Silvia De Stefano et al.
The Journal of physiology, 591(23), 5879-5893 (2013-10-09)
ClC-5 is a 2Cl(-)/1H(+) antiporter highly expressed in endosomes of proximal tubule cells. It is essential for endocytosis and mutations in ClC-5 cause Dent's disease, potentially leading to renal failure. However, the physiological role of ClC-5 is still unclear. One
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