CLCN5 Antibodies

H(+)/Cl(-) exchange transporter 5 (CLCN5)

Proton-coupled chloride transporter. Functions as antiport system and exchanges chloride ions against protons.

Important for normal acidification of the endosome lumen. May play an essential role in renal tubular function.

Gene Information

Human
Gene Name:	CLCN5
Uniprot:	P51795
Entrez:	1184

Family

Belongs to:
chloride channel (TC 2.A.49) family

Alternative Names

chloride channel 5; Chloride channel protein 5; Chloride transporter ClC-5; CLC5; clC-5; CLCK2NPHL2; DENTSNPHL1; H(+)/Cl(-) exchange transporter 5; hCIC-K2; hClC-K2; nephrolithiasis 1 (X-linked); nephrolithiasis 2, X-linked; XLRH; XRN

Molecular Weight

Mass (kDA):
83.147 kDA

Genomic Context

Human
Location:	Xp11.23
Sequence:	X; NC_000023.11 (49922596..50099235)

Tissue Specificity

Kidney. Moderately expressed in aortic vascular smooth muscle and endothelial cells, and at a slightly higher level in the coronary vascular smooth muscle.

Subcellular Localizations

Golgi apparatus membrane; Multi-pass membrane protein. Endosome membrane; Multi-pass membrane protein. Cell membrane; Multi-pass membrane protein.

Diseases

• Dent's Disease
• Proteinuria Of Undiagnosed Cause
• Hypercalciuria
• Nephrocalcinosis
• Nephrolithiasis
• Kidney Diseases
• Kidney Failure
• Kidney Calculi
• Rickets
• Renal Tubular Disorder
• Fanconi Syndrome
• Genetic Diseases, X-linked
• Hypophosphatemic Rickets

• Hypophosphatemia
• Oculocerebrorenal Syndrome
• Renal Insufficiency
• Phosphate Diabetes
• Aminoaciduria
• Acidosis
• Familial Hypophosphatemia

Interacting Proteins

• KIF3B

Pathways

• Endocytosis
• Excretion
• Transport
• Pathogenesis
• Translation
• Receptor-mediated Endocytosis
• Reverse Transcription
• Cell Proliferation
• Glycosylation
• Localization
• Glomerular Filtration
• Secretion
• Cell Adhesion

• Gene Silencing
• Osteoblast Differentiation
• Cell Cycle Phase
• Immune Response
• Regulation Of Gene Expression
• Chondrocyte Differentiation
• Intestinal Absorption

PTMs

• Glycosylation

• Biotinylation

• Hydroxylation

For details, visit:
bosterbio.com/bosterbio-gene-info-cards/CLCN5

Best Uses Of The CLCN5 Marker

Anti-Claudin-5/CLDN-5 Marker is a highly specific antibody that reacts with human Claudin-5. It has been validated for use in immunofluorescence, IHC, and ICC. This product is designed to recognize Human and Mouse Claudin-5 and is a high-quality antibody for use in research. Cldn5 is a vital EC element and has been linked with paracellular permeability and BBB. The BBB may also be affected by Cldn3 or -12.

Immunohistochemistry

Boster Bio specializes on the production of picogram sensitivity ELISA kit kits and IHC-optimized monoclonal antibodies. Boster Bio's antibodies have been tested against over 250 different tissues. The company also uses validated antisera that are derived from known positive and negative samples to guarantee high specificity and affinity. Boster Bio's anti CLCN5 antibody will work in any laboratory, regardless of whether you are trying to detect CLCN5 protein or other recombinant proteins.

The CLCN5 Marker is an excellent tool for biomarker research. It is capable of identifying age-associated genes and other proteins within cells. This marker is particularly useful in studies that are aimed at the prevention or treatment of Alzheimer's. These diagrams show the best uses for the CLCN5 gene. These examples do not represent all possible uses of the CLCN5 gene.

Immunohistochemistry methods

As part of their diagnostic work, pathologists often use immunohistochemistry (IHC) to determine whether a patient has cancer. Immunohistochemistry uses immunohistochemistry to identify specific molecules within cells, called markers. Pathologists can identify subtypes and types of cancer by examining the patterns of these molecules. A biopsy must first be taken from a tumor to perform this test. The type of cancer and the location of the tumor will determine which biopsy procedure is used.

This assay can be performed using two types of immunohistochemistry. In the first type, both the primary and secondary antibodies must come from the exact same species. The second type of method has the primary antibody unlabeled and the secondary antibody labeled using a fluorescent dye. You can also use other types of dyes. Patients will have their own preferences about the optimal titer for each test.

Blocking buffers are the best immunohistochemistry technique for the CLCN5 marker. Blocking buffers help minimize background staining. Non-fat dry milk and bovine serum albumin are common blocking buffers. Some buffers include gentle surfactants, which allow for easier wetting. These techniques are highly sensitive for the CLCN5 mark. The CLCN5 antibody was validated in several cases and is available to scientists worldwide.

Direct immunohistochemistry and Enzymatic immunohistochemistry are the most popular. These methods use reagents that allow for visualization of a target protein within a tissue section. Using these reagents, a pathologist can visualize microvessels within a tumor. The anti-F VII RAg stain, however, is not sensitive to CLCN5 markers and is not recommended for sensitive diagnosis.

There are many immunohistochemistry procedures that can be used in order to analyze CLCN5 within human tissues. The most commonly used immunohistochemistry method is the use of rabbit CD105 anti-mouse antibody or rat CD105. However, the anti-F VIII RAG method can be used to detect CLCN5 in various tumor samples. Regardless of the method used, one thing is for sure, the CLCN5 protein is highly immunoreactive in human and mouse tissue.

Antibodies for detection

A Nature Communications study revealed that the CLCN5 gene was found in oocytes of six probands, three affected individuals and six unaffected controls. Western blots can detect this marker. To confirm the mutation of CLCN5, a CLCN5 antibody against its carboxyterminal amino acid residues was raised.

A synthetic peptide containing aminos 30-51 was combined to a carrier peptide to make a CLCN5 antibody. The CLCN5 human gene has homologous sequences with the rat. It functions as an antiport system by exchanging protons with chloride ions. CLCN5 is believed to play a role in renal tubular function. A variety of renal diseases can result from deficiencies in CLCN5, such as hypophosphatemic rickets or X-linked.

To detect ClC-5, HEK293 cell lines were transplanted onto glass coverslips with 4% paraformaldehyde. After 18 minutes in citrate buffer, cells were permeated with 0.1% Triton X100. The BMDMs were then treated with antibodies to the CLCN5 marker. The samples were then stained with primary antibodies that target the CLCN5 protein C-terminus. Secondary antibodies were also used for ClC-5 detection in lysosomes.

Clinical applications

CLCN5Alu has been identified by molecular analysis of CLCN5 gene. This gene contains a high score ESE motif bound by SRp40. The distance between SF2/ASF's motif and CLCN5's corresponding high score ESE motif is increased when the Alu component is introduced. Some studies have shown that functional ESEs are found in specific places at splice sites.

The CLCN5 coding region was amplified in three overlapping RT-PCR reactions. The RNA taken from a healthy individual was then used in place of RNA isolated from the kidney biopsies. Because CLCN5's splicing pattern is the same in blood cells as leukocytes, it was assumed blood cells would be a suitable source for RNA. The gene-specific marker was created and can be used to diagnose clinical conditions.

CLCN5 mutation causes the open reading frame to be altered and adds 12 codons codon 644, which leads to a premature stop codon. The predicted truncation removes 102 amino acids from the carboxy terminus, and the second CBS motif and PY motif are eliminated. These two motifs are common in proteins that contain tandem pairs. They form Batemandomains that bind adenosine derivatives.

The CLCN5 gene was analyzed to reveal six novel mutations, four deletional frameshifts, and one nonsense mutation. Mutations in CLCN5 cause low-molecularweight proteinuria and renal phosphate loss. Some CLCN5 mutations may cause the disease not to manifest as expected. This gene could play an important role in the treatment of Dent's disease.

The CLC-5 genes are expressed in the proximal trunule in the presence of CLCN5 in the human nephron. It is also found in a-intercalated cellular structures of the collecting tube and the thick ascending branch. These tissues indicate that it plays a role in regulating the endosomal pH. CLCN-5 knockout mice also showed that it interferes in endosomal acidification. They also have impaired fluid-phase and receptor-mediated protein reabsorption. CLC-5 may play a significant role in the processing and absorption of reabsorbed substances.

CLCN5 deregulation in DRG neurons significantly decreased the S1P-induced current density. However, it did not affect the density of S1P-induced current in sensory neurons. Two chloride channels are responsible for Clcn5 contributing to the excitatory conductance sensory neurons. CLCN5 mRNA levels have decreased in DRG neurons by adenovirus gene silencing.