Product info:

The product of this gene belongs to the CLC chloride channel family of proteins. Chloride channels play important roles in the plasma membrane and in intracellular organelles. This gene encodes chloride channel 7. Defects in this gene are the cause of osteopetrosis autosomal recessive type 4 (OPTB4), also called infantile malignant osteopetrosis type 2 as well as the cause of autosomal dominant osteopetrosis type 2 (OPTA2), also called autosomal dominant Albers-Schonberg disease or marble disease autosoml dominant. Osteopetrosis is a rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. OPTA2 is the most common form of osteopetrosis, occurring in adolescence or adulthood.

Alternative names:

CLCN7; CLC-7; CLC7; OPTA2; OPTB4; PPP1R63

Species reactivity:

Human

Host:

Rabbit

Cellular localisation:

Lysosome membrane, Multi-pass membrane protein

Isotype:

IgG

Immunogen:

Recombinant fusion protein containing a sequence corresponding to amino acids 626-805 of human CLCN7 (NP_001278.1).

Positive control:

Request info at info@sobekbio.com

AA Sequence:

TAREVMSTPVTCLRRREKVGVIVDVLSDTASNHNGFPVVEHADDTQPARLQGLILRSQLIVLLKHKVFVERSNLGLVQRRLRLKDFRDAYPRFPPIQSIHVSQDERECTMDLSEFMNPSPYTVPQEASLPRVFKLFRALGLRHLVVVDNRNQVVGLVTRKDLARYRLGKRGLEELSLAQT

Purification:

Affinity purification

Molecular weight:

Calculated MW: 86kDa/88kDa

Form:

Liquid

Applications:

Request info at info@sobekbio.com

Dilution:

IF 1:50 - 1:100

Storage buffer:

Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Storage:

Store at 4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.

More info:

Email: info@sobekbio.com

Orders:

Email: orders@sobekbio.com