CHST14 Antibody

Code CSB-PA839851LA01HU
Size US$166
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Image
  • Western Blot
    Positive WB detected in: A549 whole cell lysate
    All lanes: CHST14 antibody at 4.8µg/ml
    Secondary
    Goat polyclonal to rabbit IgG at 1/50000 dilution
    Predicted band size: 43 kDa
    Observed band size: 43 kDa

  • IHC image of CSB-PA839851LA01HU diluted at 1:800 and staining in paraffin-embedded human pancreatic cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) CHST14 Polyclonal antibody
Uniprot No.
Target Names
CHST14
Alternative Names
2600016L03Rik antibody; ATCS antibody; Carbohydrate (N acetylgalactosamine 4 0) sulfotransferase 14 antibody; Carbohydrate sulfotransferase 14 antibody; CHST14 antibody; CHSTE_HUMAN antibody; D4ST-1 antibody; D4st1 antibody; Dermatan 4-sulfotransferase 1 antibody; hD4ST1 antibody; RP23-286G12.2 antibody; UNQ1925/PRO4400 antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Carbohydrate sulfotransferase 14 protein (70-133AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The CHST14 Antibody (Product code: CSB-PA839851LA01HU) is Non-conjugated. For CHST14 Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA839851LB01HU CHST14 Antibody, HRP conjugated ELISA
FITC CSB-PA839851LC01HU CHST14 Antibody, FITC conjugated
Biotin CSB-PA839851LD01HU CHST14 Antibody, Biotin conjugated ELISA
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Tested Applications
ELISA, WB, IHC
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:5000
IHC 1:500-1:1000
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Catalyzes the transfer of sulfate to position 4 of the N-acetylgalactosamine (GalNAc) residue of dermatan sulfate. Plays a pivotal role in the formation of 4-0-sulfated IdoA blocks in dermatan sulfate. Transfers sulfate to the C-4 hydroxyl of beta1,4-linked GalNAc that is substituted with an alpha-linked iduronic acid (IdoUA) at the C-3 hydroxyl. Transfers sulfate more efficiently to GalNAc residues in -IdoUA-GalNAc-IdoUA- than in -GlcUA-GalNAc-GlcUA-sequences. Has preference for partially desulfated dermatan sulfate. Addition of sulfate to GalNAc may occur immediately after epimerization of GlcUA to IdoUA. Appears to have an important role in the formation of the cerebellar neural network during postnatal brain development.
Gene References into Functions
  1. Dermatan sulfate does not appear in urine of patients with Ehlers-Danlos syndrome caused by a CHST14/D4ST1 deficiency. PMID: 28238810
  2. patients with CHST14/D4ST1 deficiency develop progressive multisystem fragility-related manifestations, establishment of a comprehensive and detailed natural history and health-care guidelines as well as further elucidation of the pathophysiology in view of future etiology-based therapy are crucial PMID: 26646600
  3. CHST14 gene mutations are associated with musculocontractural type of Ehlers-Danlos syndrome. PMID: 26373698
  4. We report on the detailed clinical characterization of two sisters with musculocontractural Ehlers-Danlos syndrome caused by a homozygous mutation in the CHST14 gene. PMID: 22581468
  5. Loss of dermatan-4-sulfotransferase 1 (D4ST1/CHST14) function represents the first dermatan sulfate biosynthesis defect, "dermatan sulfate-deficient adducted thumb-clubfoot syndrome". PMID: 21309034
  6. Musculocontractural Ehlers-Danlos Syndrome (former EDS type VIB) and adducted thumb clubfoot syndrome (ATCS) represent a single clinical entity caused by mutations in the dermatan-4-sulfotransferase 1 encoding CHST14 gene. PMID: 20842734
  7. A homozygous CHST14 (carbohydrate sulfotransferase 14) mutation in the two familial cases and compound heterozygous mutations in four sporadic cases of Ehlers-Danlos syndrome, were identified. PMID: 20533528
  8. D4ST-1 is a key enzyme and is indispensable in the formation of important functional domains in dermatan sulfate and cannot be compensated by other 4-O-sulfotransferases PMID: 19661164
  9. dermatan-4-sulfotransferase 1 has a role in adducted thumb-clubfoot syndrome PMID: 20004762
  10. D4ST-1 is encoded by a single exon located on human chromosome 15q14; type II membrane protein of 376 amino acids with a 43-amino acid cytoplasmic domain and a 316-amino acid luminal domain containing two potential N-linked glycosylation sites PMID: 11470797

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Involvement in disease
Ehlers-Danlos syndrome, musculocontractural type 1 (EDSMC1)
Subcellular Location
Golgi apparatus membrane; Single-pass type II membrane protein.
Protein Families
Sulfotransferase 2 family
Tissue Specificity
Widely expressed. Expressed at high level in pituitary gland, placenta, uterus and thyroid.
Database Links

HGNC: 24464

OMIM: 601776

KEGG: hsa:113189

STRING: 9606.ENSP00000307297

UniGene: Hs.442449

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