Recombinant Full Length Human ADAMTSL1 Protein, GST-tagged

• Cat.No.: ADAMTSL1-919HF
• Product Overview: Human ADAMTSL1 full-length ORF ( AAH30262.1, 1 a.a. - 439 a.a.) recombinant protein with GST-tag at N-terminal.

Specification

• Description: This gene encodes a secreted protein and member of the ADAMTS (a disintegrin and metalloproteinase with thrombospondin motif) family. This protein lacks the metalloproteinase and disintegrin-like domains, which are typical of the ADAMTS family, but contains other ADAMTS domains, including the thrombospondin type 1 motif. This protein may have important functions in the extracellular matrix. Alternative splicing results in multiple transcript variants encoding distinct proteins. [provided by RefSeq, Jul 2008]
• Source: In Vitro Cell Free System
• Species: Human
• Tag: GST
• Molecular Mass: 75.2 kDa
• Protein Length: 439 amino acids
• AA Sequence: MECCRRATPG TLLLFLAFLL LSSRTARSEE DRDGLWDAWG PWSECSRTCG GGASYSLRRC LSSKSCEGRN IRYRTCSNVD CPPEAGDFRA QQCSAHNDVK HHGQFYEWLP VSNDPDNPCS LKCQAKGTTL VVELAPKVLD GTRCYTESLD MCISGLCQIV GCDHQLGSTV KEDNCGVCNG DGSTCRLVRG QYKSQLSATK SDDTVVAIPY GSRHIRLVLK GPDHLYLETK TLQGTKGENS LSSTGTFLVD NSSVDFQKFP DKEILRMAGP LTADFIVKIR NSGSADSTVQ FIFYQPIIHR WRETDFFPCS ATCGGGYQLT SAECYDLRSN RVVADQYCHY YPENIKPKPK LQECNLDPCP ARWEATPWTA CSSSCGGGIQ SRAVSCVEED IQGHVTSVEE WKCMYTPKMP IAQPCNIFDC PKWLAQEWSP VTVPSFFVH
• Applications: Enzyme-linked Immunoabsorbent Assay
  Western Blot (Recombinant protein)
  Antibody Production
  Protein Array
• Storage: Store at -80 centigrade. Aliquot to avoid repeated freezing and thawing.
• Storage Buffer: 50 mM Tris-HCl, 10 mM reduced Glutathione, pH=8.0 in the elution buffer.

Gene Information

• Gene Name: ADAMTSL1 ADAMTS-like 1 [ Homo sapiens ]
• Official Symbol: ADAMTSL1
• Synonyms: ADAMTSL1; ADAMTS-like 1; C9orf94, chromosome 9 open reading frame 94; ADAMTS-like protein 1; ADAMTSR1; FLJ35283; punctin; punctin-1; ADAM-TS related protein 1; C9orf94; PUNCTIN; ADAMTSL-1; FLJ41032; FLJ46891; MGC40193; MGC118803; MGC118805; DKFZp686L03130
• Gene ID: 92949
• mRNA Refseq: NM_001040272
• Protein Refseq: NP_001035362
• MIM: 609198
• UniProt ID: Q8N6G6

Q&As

How is ADAMTSL1 protein studied in the laboratory?

ADAMTSL1 protein can be studied using a range of biochemical techniques including immunoblotting and immunohistochemistry. Animal models of ADAMTSL1-related disorders can also be used to study the molecular mechanisms underlying these diseases and to test potential therapeutic strategies.

What is the potential for ADAMTSL1 protein as a drug target?

ADAMTSL1 protein may be a potential drug target for therapies aimed at treating disorders associated with abnormal extracellular matrix composition, such as Ehlers-Danlos syndrome. Targeting this protein may help normalize the extracellular matrix and improve symptoms associated with these disorders.

How can ADAMTSL1 protein be used as a biomarker for EDS?

ADAMTSL1 protein levels in the blood or other bodily fluids may serve as a biomarker for EDS. Decreased levels of ADAMTSL1 protein are associated with certain types of EDS, such as the dermatosparaxis type. However, more research is needed to validate the specificity and sensitivity of ADAMTSL1 as a biomarker for EDS.

Are there any limitations in using ADAMTSL1 protein as a biomarker or drug target?

One limitation in using ADAMTSL1 protein as a biomarker or drug target is the limited understanding of its exact role in different tissues and diseases. Other factors such as genetic variation in the ADAMTSL1 gene, environmental factors, and other interactions with cellular processes may also affect its applicability in different populations. Additionally, more research is needed to fully validate its potential as a biomarker and drug target.

What are some diseases associated with mutations in the ADAMTSL1 gene?

Mutations in the ADAMTSL1 gene are associated with several connective tissue disorders, including dermatosparaxis-type Ehlers-Danlos syndrome, which is characterized by lax skin, joint hypermobility, and abnormal scarring. ADAMTSL1 mutations are also associated with Geleophysic dysplasia, a rare genetic disorder characterized by short stature, skeletal abnormalities, and facial dysmorphisms.

Are there any drugs or therapies targeting ADAMTSL1 protein currently in development?

At this time, there are no drugs or therapies specifically targeting ADAMTSL1 protein in development for EDS or other related conditions. However, research into potential therapeutic targets for EDS is ongoing, and future drugs or therapies may target ADAMTSL1 or related proteins.

What is the diagnostic value of ADAMTSL1 protein in connective tissue disorders?

Reduced levels of ADAMTSL1 protein in the blood or tissues have been found in individuals with dermatosparaxis-type Ehlers-Danlos syndrome, suggesting that it may serve as a diagnostic biomarker. However, further studies are needed to validate its use for diagnostic purposes.

What are some potential therapeutic targets for ADAMTSL1-related disorders?

Some potential therapeutic targets for ADAMTSL1-related disorders include the use of gene therapy to restore the production of functional ADAMTSL1 protein, the development of small molecule inhibitors or activators of ADAMTSL1 function, and the use of drugs that regulate collagen production or turnover.

Can ADAMTSL1 protein be used as a therapeutic target for EDS?

ADAMTSL1 protein may be a potential therapeutic target for treating EDS. Targeting this protein could help normalize the ECM and improve tissue structure and function in individuals with EDS. However, more research is needed to determine whether this approach is clinically feasible and effective.

What other conditions or diseases may ADAMTSL1 protein be linked to?

ADAMTSL1 protein has been implicated in several other diseases, including Marfan syndrome, aortic aneurysms, and hypertension. However, the exact role of ADAMTSL1 in these conditions is less well understood and requires further investigation.

What are the potential applications of ADAMTSL1 protein?

ADAMTSL1 protein has potential applications in research and clinical settings. It may be used as a biomarker to identify connective tissue disorders such as Ehlers-Danlos syndrome. ADAMTSL1 protein may also be a potential therapeutic target for treating disorders associated with abnormal extracellular matrix composition.

How can ADAMTSL1 protein be studied in the lab?

ADAMTSL1 protein can be studied using a variety of methods, including Western blotting, immunohistochemistry, and enzyme-linked immunosorbent assays (ELISAs). Animal and cellular models of EDS may also be used to study ADAMTSL1 function and potential therapeutic targets.

How can ADAMTSL1 protein be used as a biomarker?

A decrease in ADAMTSL1 protein levels may be indicative of connective tissue disorders such as Ehlers-Danlos syndrome. Therefore, measuring ADAMTSL1 protein levels in serum or other bodily fluids may be a useful diagnostic tool for these disorders.