ISPD Antibody (PACO46454)
- SKU:
- PACO46454
- Product Type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- ELISA
- Applications:
- IHC
- Applications:
- IF
- Antibody Type:
- Polyclonal Antibody
- Conjugation:
- Unconjugated
Description
| Antibody Name: | ISPD Antibody (PACO46454) |
| Antibody SKU: | PACO46454 |
| Size: | 50ug |
| Host Species: | Rabbit |
| Tested Applications: | ELISA, IHC, IF |
| Recommended Dilutions: | ELISA:1:2000-1:10000, IHC:1:20-1:200, IF:1:50-1:200 |
| Species Reactivity: | Human |
| Immunogen: | Recombinant Human Isoprenoid synthase domain-containing protein (1-264AA) |
| Form: | Liquid |
| Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
| Purification Method: | >95%, Protein G purified |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Non-conjugated |
 | Immunohistochemistry of paraffin-embedded human liver cancer using PACO46454 at dilution of 1:100. |
 | Immunofluorescent analysis of HepG2 cells using PACO46454 at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). |
 | Immunohistochemistry of paraffin-embedded human testis tissue using PACO46454 at dilution of 1:100. |
| Background: | Required for protein O-linked mannosylation. Probably acts as a nucleotidyltransferase involved in synthesis of a nucleotide sugar. Required for dystroglycan O-mannosylation. |
| Synonyms: | D-ribitol-5-phosphate cytidylyltransferase (EC 2.7.7.40) (2-C-methyl-D-erythritol 4-phosphate cytidylyltransferase-like protein) (Isoprenoid synthase domain-containing protein) (hISPD), ISPD |
| UniProt Protein Function: | ISPD: Required for protein O-linked mannosylation. Probably acts as a nucleotidyltransferase involved in synthesis of a nucleotide sugar. Required for dystroglycan O-mannosylation. Defects in ISPD are the cause of muscular dystrophy- dystroglycanopathy congenital with brain and eye anomalies type A7 (MDDGA7). MDDGA7 is an autosomal recessive disorder characterized by congenital muscular dystrophy associated with cobblestone lissencephaly and other brain anomalies, eye malformations, profound mental retardation, and death usually in the first years of life. Included diseases are the more severe Walker-Warburg syndrome and the slightly less severe muscle-eye- brain disease. Belongs to the IspD family. 2 isoforms of the human protein are produced by alternative splicing.Protein type: EC 2.7.7.-; TransferaseChromosomal Location of Human Ortholog: 7p21.2Molecular Function: nucleotidyltransferase activityBiological Process: axon guidance; isoprenoid biosynthetic process; protein amino acid O-linked mannosylationDisease: Muscular Dystrophy-dystroglycanopathy (congenital With Brain And Eye Anomalies), Type A, 7; Muscular Dystrophy-dystroglycanopathy (limb-girdle), Type C, 7 |
| UniProt Protein Details: | |
| NCBI Summary: | This gene encodes a 2-C-methyl-D-erythritol 4-phosphate cytidylyltransferase-like protein. Mutations in this gene are the cause of Walker-Warburg syndrome. Alternate splicing results in multiple transcript variants. [provided by RefSeq, May 2012] |
| UniProt Code: | A4D126 |
| NCBI GenInfo Identifier: | 157671913 |
| NCBI Gene ID: | 729920 |
| NCBI Accession: | NP_001094887.1 |
| UniProt Secondary Accession: | A4D126,A8MU35, H9KVB2 |
| UniProt Related Accession: | A4D126 |
| Molecular Weight: | 44,389 Da |
| NCBI Full Name: | isoprenoid synthase domain-containing protein isoform b |
| NCBI Synonym Full Names: | isoprenoid synthase domain containing |
| NCBI Official Symbol: | ISPD |
| NCBI Official Synonym Symbols: | Nip; MDDGA7; MDDGC7; hCG_1745121 |
| NCBI Protein Information: | isoprenoid synthase domain-containing protein |
| UniProt Protein Name: | Isoprenoid synthase domain-containing protein |
| UniProt Synonym Protein Names: | 2-C-methyl-D-erythritol 4-phosphate cytidylyltransferase-like protein |
| Protein Family: | Isoprenoid synthase domain-containing protein |
| UniProt Gene Name: | ISPD |
| UniProt Entry Name: | ISPD_HUMAN |
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