92002-5
SMN1 gene [Cycle Threshold #] in DBS by NAA with probe detection
Active
Term Description
The number of cycles at which the SNM1 gene was detected using real-time PCR. If the maximum number of cycles is reached without detection of the gene, this indicates the absence of SNM1 and requires follow-up evaluation for spinal muscular atrophy (SMA).
Source: Regenstrief LOINC
Part Description
LP33177-4 SMN1 gene
The SMN1 gene (survival of motor neuron 1, telomeric) [HGNC Gene ID:11117] is located on chromosome 5q13.2. This gene is part of a 500 kb inverted duplication on chromosome 5q13. This duplicated region contains at least four genes and repetitive elements which make it prone to rearrangements and deletions. The repetitiveness and complexity of the sequence have also caused difficulty in determining the organization of this genomic region. The telomeric and centromeric copies of this gene are nearly identical and encode the same protein. However, mutations in this gene, the telomeric copy, are associated with spinal muscular atrophy; mutations in the centromeric copy do not lead to disease. The centromeric copy may be a modifier of disease caused by mutation in the telomeric copy. The critical sequence difference between the two genes is a single nucleotide in exon 7, which is thought to be an exon splice enhancer. Note that the nine exons of both the telomeric and centromeric copies are designated historically as exon 1, 2a, 2b, and 3-8. It is thought that gene conversion events may involve the two genes, leading to varying copy numbers of each gene. The protein encoded by this gene localizes to both the cytoplasm and the nucleus. Within the nucleus, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs). This protein forms heteromeric complexes with proteins such as SIP1 and GEMIN4, and also interacts with several proteins known to be involved in the biogenesis of snRNPs, such as hnRNP U protein and the small nucleolar RNA binding protein. Multiple transcript variants encoding distinct isoforms have been described. [provided by RefSeq, Jul 2014] [NCBI Gene ID:6606]
Source: National Center for Biotechnology Information (NCBI) Gene
Fully-Specified Name
- Component
- SMN1 gene
- Property
- ThreshNum
- Time
- Pt
- System
- Bld.dot
- Scale
- Qn
- Method
- Probe.amp.tar
Additional Names
- Short Name
- SMN1 gene Ct DBS Qn NAA+probe
- Display Name
- SMN1 gene NAA+probe (DBS) [ThreshNum]
- Consumer Name Alpha Get Info
- SMN1 gene, Dried blood spot
Basic Attributes
- Class
- MOLPATH
- Type
- Laboratory
- First Released
- Version 2.66
- Last Updated
- Version 2.66
- Order vs. Observation
- Both
Member of these Panels
LOINC | Long Common Name |
---|---|
104191-2 | Guanidinoacetate methyltransferase (GAMT) deficiency newborn screening panel |
54089-8 | Newborn screening panel American Health Information Community (AHIC) |
92005-8 | Spinal muscular atrophy newborn screening panel |
Language Variants Get Info
Tag | Language | Translation |
---|---|---|
es-ES | Spanish (Spain) | Gen SMN1: Synonyms: Cuantitativo |
es-MX | Spanish (Mexico) | Gen SMN1: |
fr-FR | French (France) | SMN1 gène: |
it-IT | Italian (Italy) | SMN1, gene: Synonyms: Gene SMN1 Limite di soglia Patologia molecolare Punto nel tempo (episodio) Sangue Sonda con amplificazione Sonda con amplificazione del target Sonda di DNA Spot sangue secco |
nl-NL | Dutch (Netherlands) | SMN1-gen: Synonyms: probe.amp.tar SMN1 gen |
tr-TR | Turkish (Turkey) | SMN1 geni: Synonyms: Eşik Değer |
zh-CN | Chinese (China) | SMN1 基因: Synonyms: BCD541; |
Example Units
Unit | Source |
---|---|
{Ct_value} | Example UCUM Units |
LOINC Terminology Service (API) using HL7® FHIR® Get Info
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- CodeSystem lookup
- https:
//fhir.loinc.org/CodeSystem/$lookup?system=http: //loinc.org&code=92002-5
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