Thermo Fisher Scientific GCS1 Polyclonal Antibody
GCS1 단백질을 인식하는 Rabbit Polyclonal Antibody로 Western blot 및 ELISA에 사용 가능. Human, Mouse, Rat 시료 반응. Affinity Chromatography로 정제된 액상 항체로, PBS/glycerol buffer에 보관. 연구용 전용 제품.
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Applications
| Application | Tested Dilution |
|---|---|
| Western Blot (WB) | 1:500–1:2,000 |
| ELISA | 1 µg/mL |
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human, Mouse, Rat |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Immunogen | Recombinant fusion protein containing a sequence corresponding to amino acids 60–320 of human MOGS (NP_0062932) |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 0.70 mg/mL |
| Purification | Affinity Chromatography |
| Storage Buffer | PBS, pH 7.3, with 50% glycerol |
| Contains | 0.01% thimerosal |
| Storage Conditions | -20°C, Avoid Freeze/Thaw Cycles |
| Shipping Conditions | Wet ice |
| RRID | AB_2805092 |
Product Specific Information
Immunogen sequence:
RWVLAWYRAR RAVTLHSAPP VLPADSSSPA VAPDLFWGTY RPHVYFGMKT RSPKPLLTGL MWAQQGTTPG TPKLRHTCEQ GDGVGPYGWE FHDGLSFGRQ HIQDGALRLT TEFVKRPGGQ HGGDWSWRVT VEPQDSGTSA LPLVSLFFYV VTDGKEVLLP EVGAKGQLKF ISGHTSELGD FRFTLLPPTS PGDTAPKYGS YNVFWTSNPG LPLLTEMVKS RLNSWFQHRP PGAPPERYLG LPGSLKWEDR GPSGQGQGQF L
Positive Samples: U-87MG, LO2, BxPC-3, OVCAR-3, Mouse Brain, Mouse Liver, Mouse Pancreas, Mouse Testis, Rat Liver
Cellular Location: Endoplasmic reticulum membrane, Single-pass type II membrane protein
Target Information
GCS1 cleaves the distal α1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner.
Defects in GCS1 cause type IIb congenital disorder of glycosylation (CDGIIb), also known as glucosidase I deficiency.
This syndrome is characterized by generalized hypotonia, hypomotility, dysmorphic features (prominent occiput, short palpebral fissures, retrognathia, high arched palate), generalized edema, and hypoplastic genitalia.
Symptoms include hepatomegaly, hypoventilation, feeding problems, and seizures.
The clinical course is progressive, with survival typically limited to a few months.
⚠ WARNING: This product can expose you to chemicals including mercury, which is known to the State of California to cause birth defects or other reproductive harm.
For more information, visit www.P65Warnings.ca.gov.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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